cord-000121-duwfxeyt	2008	title: Prevalence of human herpesvirus 6 antibodies and DNA in allogeneic stem cell transplant patients: two-year single centre experience INTRODUCTION: Human herpesvirus 6 (HHV-6) has been recognized as a potentially significant pathogen in hemopoietic stem cell transplant (HSCT) recipients. In a recent study we summarized retrospective results of the determination of HHV infection status in allogeneic stem cell transplant recipients. As infections with HHV-6 are rarely accompanied by clinical symptoms, our first aim was to compare HHV-6 infection status, measured as viral DNA presence in the blood in the post-transplantation period and patients'' anti-HHV-6 serological status by detection of IgG and IgM antibodies. In the two patients who had a single HHV-6-positive blood sample, viral DNA was detected at a later time. High levels of human herpesvirus 6 DNA in peripheral blood leucocytes are correlated to platelet engraftment and disease in allogeneic stem cell transplant patients
cord-003866-3gwbc7z9	2019	[38] MRSA was detected in the oral cavity following HSCT, especially during OM peak severity HSCT CT chemotherapy, CONS coagulase-negative staphylococci, HSCT hematopoietic stem cell transplantation, MRSA methicillin-resistant staphylococcus spp., OM oral mucositis When pattern recognition receptors (PRRs) expressed by cells of the innate immune system within the mucosa (e.g., macrophages, neutrophils, and dendritic cells) are exposed to CRAMPS, this results in the release of cytokines that act locally, and also may have distant effects by activating intracellular and intercellular signaling loops [64] . [70] Treatment of dental pathologies and reducing oral microbial load resulted in shorter duration of OM HSCT Khan and Wingard [54] Presence of oropharyngeal mucositis is an independent risk factor for bacteremia in neutropenic patients HSCT, non-Hodgkin lymphoma, leukemia Head and neck cancer FN febrile neutropenia, HSCT hematopoietic stem cell transplantation infrequent conversion of chronic dental disease to an acute state during CT and conclude that there is no need for the treatment of "asymptomatic" chronic dental infections prior to CT or conditioning therapy for HSCT [62, 108] .
cord-004675-n8mlxe7p	2019	However, the mean infusion rate per site was similar between patients aged <18 years ( XMEN disease (X-linked Immunodeficency with Magnesium defect, Epstein-Barr virus infection and Neoplasia) is a primary immune deficiency caused by mutations in MAGT1 and characterized by chronic infection with Epstein-Barr virus (EBV), EBV-driven lymphoma, CD4 T-cell lymphopenia, and dysgammaglobulinemia. We present the case of a 1-year old Hispanic infant with a pathogenic variant in MAGT1 gene that clinically manifested with early Pneumocystis jirovecii and cytomegalovirus (CMV) interstitial pneumonia, and EBV chronic infection with good response to intravenous immunoglobulins supplementation without hematopoietic stem cell transplantation or gene therapy. Chief, Laboratory of Clinical Immunology and Microbiology, IDGS, DIR, NIAID, NIH, Bethesda, MD, USA Hypomorphic Recombination Activating Gene 1 (RAG1) mutations result in residual T-and B-cell development in both humans and mice and have been found in patients presenting with delayed-onset combined immune deficiency with granulomas and/or autoimmunity (CID-G/AI).
cord-005446-jr3yj4o2	2003	title: Low molecular weight heparin for the prevention of hepatic veno-occlusive disease (VOD) after hematopoietic stem cell transplantation: a prospective phase II study We evaluated 40 patients undergoing high-dose chemo/ radiotherapy (HDCT) and hematopoietic stem cell transplantation (HSCT) (allogeneic (22) , autologous (18) ) to determine the safety and feasibility of administering low molecular weight heparin (LMWH) as hepatic veno-occlusive disease (VOD) prophylaxis. We evaluated 40 patients undergoing high-dose chemo/ radiotherapy (HDCT) and hematopoietic stem cell transplantation (HSCT) (allogeneic (22) , autologous (18) ) to determine the safety and feasibility of administering low molecular weight heparin (LMWH) as hepatic veno-occlusive disease (VOD) prophylaxis. Daily patient evaluation while receiving dalteparin included patient weight, oxygen saturation, liver size, presence of right upper quadrant (RUQ) abdominal pain, ascites or pleural effusions, complete blood count (CBC), serum creatinine, assessment of bleeding and GVHD; adverse events, grading of RRT and the number of red cell and platelet transfusions were recorded; liver function tests (bilirubin, AST, ALT, alkaline phosphatase), INR, PTT and fibrinogen were measured twice weekly.
cord-005448-5fznfp5p	2010	title: CD34(+) cell dose and establishment of full donor chimerism at day +100 are important factors for survival with reduced-intensity conditioning with fludarabine and melphalan before allogeneic hematopoietic SCT for hematologic malignancies The combination of fludarabine and melphalan as a reduced-intensity conditioning (RIC) regimen extends allogeneic hematopoietic SCT (HSCT) as a therapeutic option for elderly or frail patients with relapsed, refractory or other high-risk hematologic malignancies. Factors analyzed for association with survival included demographic and disease factors (age at transplant, sex, lymphoid vs myeloid disease, karyotype and blast percentage in those with MDS and leukemia, disease status at transplant, previous HSCT, and previous chemotherapy for solid tumors), donor/graft factors (CD34 þ cell dose, graft source, related vs unrelated graft, degree of HLA match, CMV serostatus and ABO match), and post transplant factors (time to neutrophil, lymphocyte and platelet engraftment, day þ 100 chimerism status, development of and severity of acute GVHD, development of and severity of chronic GVHD).
cord-005453-4057qib7	2019	To compare the safety and efficacy of prophylactic DLI for prevention of relapse after allogeneic peripheral blood stem cell transplantation from haploidentical donors (HID-SCT) and matched-sibling donors (MSD-SCT) in patients with very high-risk acute myeloid leukemia (AML), we performed a retrospective, observational cohort study enrolled in 21 HID-SCT and 13 MSD-SCT recipients. The aim of this study is to identify the prognostic impact of pre-transplant TIM3 levels on early and late transplant related complications as well as post-transplant relapse and survival Methods: A total of 177 hematopoietic stem cell transplantation (HSCT) recipients with an initial diagnosis of acute leukemia [median age: 36(16-66) years; male/ female: 111/66] were included in the study.
cord-005460-ezrn8cva	2017	Still the optimal combination of immunosuppressive agents with PTCy should be elucidated for different types of SCTs. We report the 2-year update of the prospective NCT02294552 single-center trial that evaluated risk-adapted graft-versushost disease (GVHD) prophylaxis with PTCy in related, unrelated and haploidentical SCTs. 200 adult patients (median age 32 y.o., range: 18-62) with hematologic malignancies, including AML (47.5%), ALL (26.5%), CML (10.5%), MDS (4%), and lymphomas (11.5%), were enrolled in the study. Long-term follow-up from the prospective randomized phase III multicenter trial comparing a standard GvHD prophylaxis with cyclosporine A and methotrexate with or without additional pretransplant ATLG (Grafalon, previously ATG-FRESENIUS S) (given 20 mg/kg/day, days − 3 to − 1) in unrelated donor hematopoietic cell transplantation after myeloablative conditioning resulted in a significant reduction of acute and chronic GvHD without compromising relapse rate and survival [1, 2, 3] .
cord-005478-5iu38pr6	2019	There were some differences among groups: patients in group-1 were younger (median age 46 years, p< 0.02) were transplanted in more recent year (2015, p< 0.001), received more frequently a regimen based on TBF (thiotepa, fludarabine and busulfan) (83%, p< 0.001) and bone marrow (BM) as source of stem cells (77%, p< 0.001), with no ATG (100%, p< 0.001). Clinical Trial Registry: NCT01217723 Disclosure: None of the Authors have any conflicts of interest to declare O105 Immune reconstitution -based score at diagnosis of CGVHD predicts GVHD severity and overall-survival: A novel prognostication tool for GVHD treatment tailoring Background: Allogeneic stem cell transplantation (HSCT) survivors are at a relevant risk of developing chronic GvHD (cGvHD), which importantly affects quality of life and increases morbidity and mortality.
cord-005480-yg7salqt	2008	Standard NIH or Eurolupus cyclophosphamide (CY) protocols and mycophenolate Mofetil (MMF) as induction therapy in severe BILAG A SLE is still associated with 20 % failure, 50% relapse and 10% to 15 % death at 10 years In the absence of a single standard treatment worldwide for refractory SLE, phase I-II studies analysed the use of: a) rituximab (anti CD20 mAb) in more than 1 000 patients showing complete to partial early response around 100% with relapse in 50 to 60% of the cases; b) autologous Hematopoietic Stem Cell Transplantation (HSCT) since 1997 under the auspices of the joined EBMT-EULAR working party, reporting durable remission with reduced or no immunosuppressive drug requirement in 66%, one-third of whom later relapsed to some degree with a 74 ± 7% (n= 62/79) overall survival at 5 years for SLE among the 863 HSCT procedures registered: in 2007 in the EBMT data base.
cord-005482-v5iayczy	2016	Material (or patients) and methods: Case Report 23-year-old male patient who underwent allogeneic hematopoietic stem cell tranplantation sixteen months ago, admitted to bone marrow transplantation clinic for shortness of breath. Material (or patients) and methods: Data sets of 436 consecutive patients who underwent allogeneic HSCT at a single center (University of Rostock) from 1998 to 10/2015 were analysed regarding their early mortality rates (d+30, d+60 Introduction: Mobilization and collection of CD34-positive stem cells for subsequent high-dose chemotherapy constitutes a standard approach in myeloma patients achieving a good remission after induction chemotherapy. Outcomes of high grade gastrointestinal gvhd post-hsct in children Material (or patients) and methods: This is a retrospective analysis of 28 pediatric patients presented with a clinical diagnosis of stage 3 and 4 acute GVHD of the GIS who were selected from allogeneic hematopoetic stem cell transplantation (HSCT) performed.
cord-005487-vac061r8	2010	We retrospectively analyzed 1257 patients (pts), 755 children (age≤18) and 502 adults, receiving fi rst single (n = 1080) or double UCBT (n = 177) in EBMT centers, between 1995 and 2009 , for malignant and non-malignant diseases, who survived at least 100 days from transplantation with neutrophils recovery and without relapse or autologous reconstitution. Prochymal® improves response rates in patients with steroid-refractory acute graft-versus-host disease involving the liver and gut: results of a randomized, placebo-controlled, multicentre phase III trial in GvHD P.J. Martin (1) , J.P. Uberti (2) Background and methods: Steroid-refractory acute GVHD (SR-GVHD) remains a signifi cant and life-threatening complication of allogeneic hematopoietic cell transplantation (HCT). A. Nagler (1) Background: Allogeneic transplantation of hematopoietic stem cells (allo-SCT) from an HLA-matched related (MRD) or unrelated donor (URD) is a curative option for patients (pts) with high-risk hematological disease (HRHD).
cord-006466-e1phpqes	2018	Whole exome sequencing revealed a heterozygous mutation, previously reported (c.1425+1G>T) Conclusions: In summary, this report emphasizes the suspicion of a combined immunodeficiency in the presence of multiple abscesses by Mycoplasma, the usefulness of rDNA 16s in order to achieve proper Objectives: We describe a 15-year-old male patient with novel heterozygous mutation of EP300 gene; his first manifestations were initially characterized by infections, cytopenia and hypogammaglobulinemia suggesting a Common Variable Immunodeficiency (CVID), but later on, persisting lymphopenia was suggestive of a combined immunodeficiency. Conclusions: Close monitoring of immune function in early life for patients with CHH and CID as well as the availability of suitable donors assists in determining management, including HSCT Introduction/Background: Leukocyte Adhesion Deficiency (LAD) represents a group of distinct inherited disorders, which inhibit the normal extravasation of neutrophils and their recruitment to sites of infection or inflammation.
cord-006492-pc1vayyl	2016	BACKGROUND: X-linked inhibitor of apoptosis protein (XIAP) deficiency is a rare immunodeficiency that is characterized by recurrent hemophagocytic lymphohistiocytosis (HLH) and splenomegaly and sometimes associated with refractory inflammatory bowel disease (IBD). Although hematopoietic stem cell transplantation (HSCT) is the only curative therapy, the outcomes of HSCT for XIAP deficiency remain unsatisfactory compared with those for SLAM-associated protein deficiency and familial HLH. CONCLUSION: The RIC regimen and HLH control might be important factors for successful HSCT outcomes, with improved IBD, in patients with XIAP deficiency. The transplantations were performed in different institutions, but all conditioning regimens were RIC, except that for patient 1, who was given an intermediate intensity regimen, but died of HLH and acute respiratory distress syndrome on day 27 post-HSCT, in which virus infection or reactivation might not be involved. Reduced-intensity conditioning hematopoietic cell transplantation is an effective treatment for patients with SLAMassociated protein deficiency/X-linked lymphoproliferative Disease type 1
cord-006856-b1w25ob5	2005	Egr-1 and hypoxia-inducible factor-1 (HIF-1) gene expression was examined in left ventricular biopsies of explanted failing hearts in 28 ICM and 42 DCM patients, as well as in 12 donor grafts before reperfusion (control), at 10, 30, 60 minutes after reperfusion, and at 1, 2, 3, 4, 6, 12 posttransplant weeks, using real-time RT-PCR. The risk of transplant-related mortality (TRM) due to graft-versushost disease (GvHD) is higher in male recipients of female stem cells compared with female patients receiving a graft from a female donor. We therefore analyzed a single-center cohort of 72 high-risk patients transplanted with a related or unrelated stem cell graft after nonmyeloablative conditioning for outcome (acute and chronic GvHD, TRM, relapse, and survival). Four patients between the age of 34 and 44 years underwent allogeneic peripheral blood stem cell (PBSC) transplantation (SCT) from HLA-identical sibling or unrelated donors at our institution.
cord-007788-09t52zix	2017	Some other relatively rare complications are also covered here: haemorrhagic cystitis (HC), endothelial damage (ED) syndromes including engraftment syndrome (ES), idiopathic pneumonia syndrome (IPS), diffuse alveolar haemorrhage (DAH), transplant-associated microangiopathy (TAM) and sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD). Oral damage may be a hallmark of graft versus host disease (GvHD) in patients following allogeneic stem cell transplantation, and the presence of lichenoid hyperkeratotic plaques (diagnostic sign), gingivitis, mucositis, erythema, pain, xerostomia and ulcers may indicate GvHD. The increased risk of infections in patients undergoing haematopoietic stem cell transplantation (HSCT) is well known, and infection is a leading cause of morbidity and mortality. Differential diagnoses will need to be excluded by assessing risk factors, symptoms and lab tests since liver dysfunction can also be seen in sepsis, viral infection, graft versus host disease (GvHD) and iron overload and as a side effect from many of the drugs used in the HSCT setting.
cord-007791-nzjm6zyq	2018	These distinct but related pathways of inflammation culminate in the recruitment of immune cells to the lung leading to tissue damage and dysfunction (Cooke and Yanik 2016) Incidence -The strict methodology required to establish IPS diagnosis and the increased use of RIC have reduced its incidence of 20% to 25% observed 20 years ago (at that time IPS was called idiopathic pneumonia) -This reduction runs in parallel of the improvement in the diagnostic methodologies to detect infectious pathogens. A recent prospective study showed that among 198 patients included after day +100, the cumulative incidence of LONIPC is 20%, and that of BOS is 11% at 3 years among allo-HSCT recipients (Bergeron et al. Late-onset non-infectious pulmonary complications following allogeneic hematopoietic stem cell transplantation in children Bronchiolitis obliterans syndrome (BOS), bronchiolitis obliterans organizing pneumonia (BOOP), and other late-onset noninfectious pulmonary complications following allogeneic hematopoietic stem cell transplantation
cord-009997-oecpqf1j	2018	Completed cranial radiation and proceeded to allogeneic stem cell transplant with unrelated cord marrow donor and is disease free at approximately day +200.Case 2: 5 year-old female diagnosed with FLT3 and MLL negative AML and completed treatment per COG AAML1031 study on the low risk arm without Bortezomib. Design/Method: This study was a retrospective chart review that included patients 3 to 23 years old with sickle cell disease type SS and S 0 followed at St. Christopher''s Hospital for Children. Background: Hydroxyurea, chronic blood transfusion, and bone marrow transplantation can reduce complications, and improve survival in sickle cell disease (SCD), but are associated with a significant decisional dilemma because of the inherent risk-benefit tradeoffs, and the lack of comparative studies. Brown University -Hasbro Children''s Hospital, Providence, Rhode Island, United States Background: Despite clinical advances in the treatment of sickle cell disease (SCD) in pediatric and young adult patients, pain remains a significant source of disease-related morbidity.
cord-010130-28bt3x25	2015	RESULTS: After a median follow‐up of 23 months, cumulative incidence of viral infections was 70% (95% confidence interval [CI] 59–81) at 100 days and 77% (95% CI 67–87) at 1 year; 35 of 65 patients at risk had CMV reactivation (54%) and the rate of polyomavirus‐virus‐associated cystitis was 19% (13/70). In the present analysis, we described infectious complications after unmanipulated, T-cell replete haplo-HSCT using post-transplant Cy in 70 consecutive patients and found, aside from a high incidence of viral infections/reactivations, especially in the early posttransplant period, a quite low incidence of late bacterial infections, together with a very low incidence of IFIs after day +180 (2 events in the overall 11 observed). In conclusion, the present single-center data on 70 consecutive patients receiving T-cell replete haplo-HSCT with post-transplant Cy confirm a high rate of viral infections before day +100 and a lower incidence of infections afterward, suggesting a satisfactory although non-optimal immune reconstitution after this type of transplantation.
cord-014712-5u4e00q6	2014	Ege University Faculty of Medicine, Dept of Pediatric Immunology, Izmir, Turkey Ig class switch recombination deficiencies are rare PIDs (1:500,000 births) with normal or elevated serum IgM and low IgG, IgA and IgE levels, defective or normal somatic hypermutation, defective T/B cooperation (50%), intrinsic B cell defect (50%), susceptibility to bacterial infections begining from the first year of age (impaired B cell immunity) and lack of germinal centres in secondary lymphoid organs. Great North Children''s Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust, and Primary Immunodeficiency Group, Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK Even following the introduction of biologic disease modifying antirheumatic drugs (DMARDs), a small number of children suffering from severe, refractory autoimmune (AI), rheumatic and/or autoinflammatory disorders will not get into clinical remission (CR) and will potentially further suffer from multiple side-effects of combined and long-term immunosuppressive and anti-inflammatory therapies, in particular severe infections (Marodi L, Casanova JL.
cord-014976-546zaoxn	2006	In order to evaluate if malignant and non malignant hematological diseases quantitatively and qualitatively affect BM derived MSCs, bone marrow from children with acute lymphoblastic leukemia (ALL diagnosis n=9, different phases of treatment n=29, end of therapy n=10), idiopathic thrombocytopenic purpura (n=16), autoimmune neutropenia (n=12) and control patients (solid tumors without BM involvement, n=30) was harvested and the mononuclear cell (MNC) fraction isolated. Case: In our hospital a total of 3 patients with relapsed Hodgkin''s disease underwent reduced-intensity conditioning (RIC) allogeneic stem cell transplantation (allo-SCT) from an HLA-identical sibling. We report a case of a young male patient of 19 years old with aggressive MS who was treated with a high-dose immunosuppressive regimen (HDIS) using myeloablation followed by autologous blood stem cell transplantation (ASCT) that has induced a dramatic and long-lasting remission of the disease.
cord-015389-vwgai4k9	2009	This study evaluates the safety of this approach, in terms of infusion-related toxicity and hematopoietic reconstitution, in 385 consecutive autologous transplantations performed from 4/97 to 9/08 in 348 patients (median age 46; underlying disease: lymphoma in 178, myeloma in 131, acute leukaemia in 17, breast cancer in 22). Patients and methods: Eight pts after allogeneic hematopoetic stem cell transplantation (HSCT) underwent MSCs infusions (median age of pts was 11 years, male/female: 6/2) between 2006 and 2009. Akiyama Tokyo Metropolitan Komagome Hospital (Tokyo, JP) Acute graft-versus-host disease (GVHD) is one of the major factors that have infl uence on the outcomes of allogeneic hematopoietic stem cell transplantation (HSCT). Material and methods: during a 8 years period we have performed 144 stem cells transplantation in 134 patients with different hematological malignancies(AML: 74; ALL: 6; CML: 7; CLL: 1, NHL: 13; Hodgkin Diseases: 16; Multiple myelomas: 24; Aplastic anaemia: 1;Myelofi brosis:1 Ewing Sarcoma: 1; Male:78 Female 66.
cord-015922-5wwy0m2k	2008	Other prophylactic strategies commonly utilized in HSCT patients include acyclovir to prevent herpes simplex virus (HSV) and VZV reactivation, fluoroquinolones [5] to prevent gram-negative sepsis and fluconazole to prevent yeast infection. It has been suggested that EBV viral load surveillance in peripheral blood be carried out in high risk patients (those with primary EBV infection, anti-T cell antibody therapy for GVHD, HLA-mismatched or T cell-depleted HSCT recipients), with decreased immunosuppression +/− antiviral therapy (acyclovir or ganciclovir) carried out in the setting of high viral loads [1, 4, 41, 42] . Infliximab use in patients with severe graftversus-host disease and other emerging risk factors of non-Candida invasive fungal infections in allogeneic hematopoietic stem cell transplant recipients: a cohort study
cord-016932-bej10xbf	2018	Early adoptive T-cell immunotherapy studies showed that administration of allogeneic virus-specific cytotoxic T lymphocytes (vCTL) can prevent and control viral infections and reconstitute antiviral immunity to cytomegalovirus (CMV) and Epstein-Barr virus (EBV). High-affinity TCR genes can be cloned and transduced into polyclonal T cells to generate a large population of (1) Blood is obtained from donors (autologous, allogeneic, or umbilical cord blood) or is drawn or apheresis is performed to obtain a larger quantity of blood; (2) PBMCs are processed via: (a) cell selection panel using multimers with a pathogen-derived peptide associated with a type-I HLA molecule or column selection after in vitro stimulation of T cells with antigens followed by binding of IFNÉ£ or CD154-expressing T cells with antibody-coated immu-nomagnetic beads; (b) cell expansion by stimulating the PBMC with APCs produced by antigenic peptide pools, viral transduction, or nucleofection; (c) genetic modification that involves the transfer of high-affinity pathogenspecific TCRs or CARs to redirect the specificity of the T cells; and (d) polyclonal expansion of T cells for 8-14 days and arming with BiAbs directed at the pathogen of interest on one hand and the TCR on the other hand; (3) quality control and release testing; and (4) infusion into patients TCR pathogen-specific CTLs [45] .
cord-017302-xez0zso3	2019	Hematopoietic stem cell transplant (HSCT) has become an essential therapeutic modality in the treatment of malignant and non-malignant hematologic disease. Allogeneic transplants are associated with more morbidity and mortality than autologous transplants, and are further categorized based on conditioning regimen (myeloablative [MA] vs non-myeloablative [NMA]), donor-recipient relation (related vs unrelated), HLA matching (full match vs haploidentical vs mismatched), and stem cell source (bone marrow, peripheral blood, umbilical cord blood). Refinement of transplant techniques over the last 2 decades has dramatically decreased transplant-related mortality, but approximately 15% of HSCT patients require critical care [10] and earlier ICU admission has been associated with improved survival rates [11, 12] . Outcomes of stem cell transplant patients with acute respiratory failure requiring mechanical ventilation in the United States Management of respiratory viral infections in hematopoietic cell transplant recipients and patients with hematologic malignancies Bronchiolitis obliterans syndrome after allogeneic hematopoietic stem cell transplantation-an increasingly recognized manifestation of chronic graft-versus-host disease
cord-017883-6a4fkd5v	2018	There are various factors which contribute to the increased susceptibility to infections in pediatric hematology/oncology (PHO) and HSCT patients, most prominent of them being disruption of cutaneous and mucosal barriers (oral, gastrointestinal, etc.), microbial gastrointestinal translocation, defects in cell-mediated immunity, and insufficient quantities and inadequate function of phagocytes. Based upon such data in adults, the IDSA Guidelines for the Use of Antimicrobial Agents in Neutropenic Patients with Cancer state that fluoroquinolone prophylaxis should be considered for high-risk patients with prolonged severe neutropenia [20] . Though some authors suggest that antibiotic prophylaxis should be considered in children undergoing induction chemotherapy for ALL, there is currently insufficient data to inform definitive guidelines for antibiotic prophylaxis to prevent bacterial infections in pediatric oncology patients [19] [20] [21] .
cord-018209-v2crgj5w	2010	Infectious and noninfectious pulmonary complications occur in 30-60% of patients with hematological malignancy and recipients of hematopoietic stem cell transplantation (HSCT) and are associated with signifi cant morbidity and mortality [1] . This chapter will review the infectious and noninfectious pulmonary findings that have been described at autopsy in patients with hematological malignancies, including blood and bone marrow transplant recipients. Table 20 .1 lists the infectious and non-infectious pulmonary disorders reported in autopsy studies of patients with hematologic malignancy, including HSCT recipients. Several autopsy series have reported diagnostic discrepancies between premortem clinical diagnosis and postmortem autopsy findings ranging from 5% to 64% in patients with hematologic malignancy and HSCT recipients (Table 20. Infectious and noninfectious pulmonary diseases are commonly found on postmortem autopsy studies in patients with hematological malignancy and HSCT recipients. Major diagnostic discrepancies between clinical premortem diagnoses and postmortem autopsy findings have been reported in patients with hematologic malignancy.
cord-018243-hyvu9nuq	2010	This chapter will address the chronic lung complications that lead to pulmonary fibrosis and persistent organ dysfunction in each context with specific focus on hematologic malignancy patients treated using HSCT. Hematologic malignancy patients treated with chemotherapy or chest wall radiation therapy, or those who proceed to receive a HSCT may develop a wide variety inflammatory noninfectious lung disorders that ultimately may lead to pulmonary fibrosis. The diagnosis of drug-induced respiratory disease often is complex because: (1)1 patients may be exposed to several pneumo-toxic drugs concurrently or in sequence due to earlier treatment failure; (2)2 time to onset of pulmonary toxicity may be delayed, making it difficult to ascertain which agent is responsible for the pulmonary reaction; (3)3 the combination of drugs to treat malignant hematologic conditions may lead to unexpected drug interactions, producing enhanced toxicity compared with the toxicity of each agent considered separately; and (4)4 radiation therapy to the chest or TBI.
cord-018302-lmly43rd	2016	Respiratory viral infections due to respiratory syncytial virus (RSV) and human metapneumovirus (hMPV) cause infections in immunocompromised transplant patients ranging from mild upper respiratory infections to severe lower respiratory tract disease with respiratory failure. Surveillance studies of respiratory viruses from transplant centers have established the high frequency and the signifi cant clinical impact of respiratory viral infections in HSCT recipients overall [ 8 -15 , 46 , 47 ] as well as the relative importance of RSV in terms of morbidity and mortality (Table 31 -2 ). A retrospective MDACC study of confi rmed RSV infections in 280 allogeneic HSCT recipients from 1996 to 2009 utilized multivariable logistic regression to demonstrate that lack of ribavirin aerosol therapy at the upper respiratory tract disease stage was an important risk factor associated with RSV LRTI and all-cause mortality [ 99 ] .
cord-018319-tylkbh4h	2011	Historically, the most common causes of respiratory infections in cancer patients were thought to be opportunistic bacteria and fungi, but newer diagnostic methods have revealed that respiratory viruses can cause serious morbidity and mortality in such patients, including leukemia patients and hematopoietic stem cell transplant (HSCT) recipients. Many viruses are known to cause respiratory tract infections, but the most common in hospitalized cancer patients are influenza viruses, respiratory syncytial virus (RSV), and parainfluenza viruses (PIV) [1, 2] . Although the combination of ribavirin and intravenous immunoglobulin (IVIG) or palivizumab has not been evaluated in a randomized trial, it is sometimes used in severely ill patients with RSV pneumonia, especially HSCT recipients, given that they have high mortality rates from this infection [3, 11, 14] . However, because other viruses can produce the same syndrome and influenza infection can produce other respiratory syndromes, a confirmatory test detecting the virus or viral antigens in nasal washes, throat swabs, respiratory tract secretions, or bronchoalveolar lavage specimens is needed in sporadic cases and in immunocompromised patients.
cord-018339-tyrlpl94	2019	A recent prospective study to evaluate the epidemiology of late non onset noninfectious complications after allogenic stem cell transplant reported a cumulative incidence of BOS 36 months posttransplant at 10.7% [9] . In a study of 9550 patients of post-allogenic HCST recipients, HLA disparity, female-to-male HSCT, and peripheral blood stem cell transplant (PBSCT) were associated with an increased risk of developing OP. Association between acute and chronic graft-versus-host disease and bronchiolitis obliterans organizing pneumonia in recipients of hematopoietic stem cell transplants Concurrent treatment with a tumor necrosis factor-alpha inhibitor and veno-venous extracorporeal membrane oxygenation in a post-hematopoietic stem cell transplant patient with idiopathic pneumonia syndrome: a case report Incidence, clinical features, and risk factors of idiopathic pneumonia syndrome following hematopoietic stem cell transplantation in children Bronchiolitis obliterans syndrome (BOS), bronchiolitis obliterans organizing pneumonia (BOOP), and other late-onset noninfectious pulmonary complications following allogeneic hematopoietic stem cell transplantation
cord-018906-03nynqtq	2014	After hematopoietic stem cell transplant (HSCT), up to 60 % of patients develop pulmonary complications. In spite of antibacterial, antiviral, and antifungal prophylaxis, reduced host defenses render the HSCT patient vulnerable to pulmonary and other infections in the early weeks and even months post-transplantation. This chapter suggests an integrative approach followed by a description of the most common pulmonary syndromes seen in HSCT patients, including diffuse alveolar hemorrhage (DAH), idiopathic pneumonia syndrome (IPS), bronchiolitis obliterans syndrome (BOS), and cryptogenic organizing pneumonia (COP). Two-year mortality after hematopoietic stem cell transplant (HSCT) has been estimated using a pre-transplantation assessment of mortality (PAM) score which incorporates spirometry and diffusing capacity variables in combination with the presence of renal and hepatic dysfunction, conditioning regimen, and disease risk. Diffuse alveolar hemorrhage (DAH) is a subset of pulmonary hemorrhage that can develop in up to 5 % of all post-HSCT recipients with mortality rates ranging between 50 and 80 % based on the two largest case series.
cord-024651-578c9ut5	2020	Abstract/Case Report Text Introduction: Mutations in the gene encoding signal transducer and activator of transcription 3 (STAT3) cause autosomal dominant hyperimmunoglobulin E syndrome (AD-HIES) characterized by recurrent skin and sinopulmonary infections, atopic dermatitis, and elevated serum immunoglobulin E (IgE) levels. Objective: The purpose of this study is to increase awareness and improve diagnosis of primary immune deficiency (PID) in the heterogenous group of patients with autoimmune cytopenia (AIC) by identifying clinical characteristics and laboratory biomarkers that distinguish those with underlying PID, disease activity and guide mechanism-based targeted therapy. 7 Chief, Laboratory of Clinical Immunology and Microbiology/National Institute of Allergy and Infectious Diseases, NIAID/National Institutes of Health, NIH Abstract/Case Report Text We have previously used the artificial thymic organoid (ATO) system, based on the 3D aggregation and culture of a delta-like canonical Notch ligand 4-expressing stromal cell line (MS5-Dll4) with CD34+ cells, to study T cell differentiation from CD34+ cells obtained from patients carrying defects that are intrinsic to hematopoietic cells (RAG1-2, AK2, IL2RG) or that affect thymus development (DiGeorge syndrome).
cord-030921-wydk9p93	2020	We report our experience with nine pediatric patients with nonmalignant diseases who were transplanted from a haploidentical donor with PT-Cy. From 2015 to 2019, nine children with nonmalignant diseases underwent haploidentical HSCT with PT-Cy, two as a second transplant and seven as primary grafts after upfront serotherapy and busulfan-based myeloablative conditioning. Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative treatment option for children with a variety of genetic nonmalignant disorders including osteopetrosis, bone marrow failure, and immune deficiencies [1] [2] [3] . Haploidentical HSCT is a readily available alternative option for patients who do not have a matched donor, but engraftment failure, high rates of graft-versus-host disease (GvHD), delayed immune reconstitution and other posttransplant complications are of significant concern. recently reported 27 haploidentical HSCT with PT-Cy for pediatric nonmalignant diseases using a busulfan-based myeloablative conditioning regimen with upfront alemtuzumab showing a high engraftment rate (24 of 27 patients engrafted) and a 2 years overall survival rate of 77.7% [6] .
cord-254183-98o0dssj	2013	In this review, we first discuss viral diagnostics and the developing field of viral discovery and then focus on rare and emerging viruses in the transplant population: human T-cell leukemia virus type 1; hepatitis E virus; bocavirus; KI and WU polyomaviruses; coronaviruses HKU1 and NL63; influenza, H1N1; measles; dengue; rabies; and lymphocytic choriomeningitis virus. Detection and reporting of such rare pathogens in transplant recipients is critical to patient care and improving our understanding of posttransplant infections. In a multicenter review of 85 cases of acute HEV infection, 65.9% of the solid organ transplant (SOT) recipients developed chronic hepatitis of whom 14.3% developed cirrhosis. Cases of lymphocytic choriomeningitis virus (LCMV) transmitted through organ transplantation (4 clusters, including 14 cases and 11 deaths) document the ability of this pathogen to cause severe disease in the immunocompromised host [10, [49] [50] [51] . Human T-cell leukemia virus type I-associated myelopathy following living-donor liver transplantation
cord-259625-8lripsf7	2019	title: Incidence, risk factors, and outcome of pulmonary invasive fungal disease after respiratory virus infection in allogeneic hematopoietic stem cell transplantation recipients BACKGROUND: There is growing evidence that community‐acquired respiratory virus (CARV) increases the risk of pulmonary invasive fungal disease (IFD) in the allogeneic hematopoietic stem cell transplantation (allo‐HSCT) setting. Generalized estimating equation model identified 4 risk factors for IFD: ATG‐based conditioning regimen [odds ratio (OR) 2.34, 95% confidence interval (CI) 1.05‐5.2, P = .038], CARV lower respiratory tract disease (OR 10.6, 95% CI 3.7‐30.8, P < .0001), CARV infection during the first year after transplant (OR 5.34, 95% CI 1.3‐21.8, P = .014), and corticosteroids during CARV (OR 2.6, 95% CI 1.1‐6.3, P = .03). Abbreviations: ALC, absolute lymphocyte count; Allo-HSCT, allogeneic hematopoietic stem cell transplantation; ANC, absolute neutrophil count; ATG, antithymocyte globulin; CARV, communityacquired respiratory virus; GvHD, graft-versus-host disease; IFD, invasive pulmonary infectious fungal disease; IS, immunosuppressants; LRTD, lower respiratory tract disease.
cord-261827-uprv8a2k	2016	title: Novel treatment of severe combined immunodeficiency utilizing ex-vivo T-cell depleted haploidentical hematopoietic stem cell transplantation and CD45RA+ depleted donor lymphocyte infusions BACKGROUND: Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative treatment available for severe combined immunodeficiency (SCID); although, there is a high incidence of severe infections and an increased risk of graft-versus host-disease (GvHD) with HSCT. Haploidentical HSCT protocols utilizing extensively ex vivo T-cell depleted grafts (CliniMACs system) have proven efficient in preventing GvHD, but cause a delay in early T-cell recovery that increases the risk of viral infections. Here, we present a novel approach for treating SCID that combines selective depletion of GvHD-inducing alpha/beta (α/β) T-cells from the haploidentical HSCT graft with a subsequent donor lymphocyte infusion (DLI) enriched for CD45RO+ memory T-cells. Unselected donor lymphocyte infusions (DLIs), which are frequently used as a "tool" to boost anti-viral immunity post-transplant, harbor a significant risk of inducing severe GvHD in the haploidentical setting [6, 7] .
cord-266359-uf1ao1x1	2015	BACKGROUND: Compared to other respiratory viruses, relatively little is known about the clinical impact of coronavirus (CoV) infection after hematopoietic stem cell transplant (HSCT) or in patients with hematologic malignancies. CONCLUSIONS: CoV is frequently detected in HSCT and hematologic malignancy patients in whom suspicion for a respiratory viral infection exists, but is less likely to progress to lower respiratory tract disease than most other respiratory viruses. The clinical significance of respiratory viruses such as influenza, respiratory syncytial virus (RSV), parainfluenza viruses (PIVs), human metapneumovirus (hMPV), rhinovirus (RhV), and adenovirus (AdV) in patients with hematologic malignancies or recipients of autologous or allogeneic hematopoietic stem cell transplant (HSCT) is well described [1] [2] [3] [4] [5] [6] [7] [8] . In conclusion, we found that CoV is detected frequently in patients with hematologic malignancies and HSCT recipients in whom suspicion for a respiratory viral infection exists, but is associated with less LRTD than other respiratory viruses except RhV/EnV.
cord-268843-zml9lbve	2018	In the Canadian province of Manitoba, our group has periodically managed young infants of Northern Cree First Nations (Aboriginal) descent presenting with early-onset and life-threatening viral, bacterial, Mycobacterial, and fungal infections, clinically resembling severe combined immune deficiency (SCID). Herein we describe the clinical presentation, immunologic features, and HSCT outcomes for the largest cohort of infants with IKBKB immune deficiency resulting from complete loss of IKKβ expression published to date. Supporting a more profound immune deficiency are the other six reported patients with IKBKB mutations, who also presented with severe bacterial, fungal and viral infections as young infants. Like IKBKB immune deficiency, patients with hypermorphic NFKBIA mutations that result in reduced degradation of IκBα, present with multiple and severe bacterial, fungal and viral infections starting at an early age, typically before 3-months.
cord-272835-6nx4f8ss	2017	Common respiratory viral infections (RVIs) are an important cause of morbidity and mortality following solid organ transplant (SOT) and hematopoietic stem cell transplant (HSCT). 18 Paulsen & Danziger-Isakov Published attributable mortality caused by LRTI in HSCT from a respiratory virus varies, up to 28% to 30% in some reports, most commonly involving influenza, RSV, adenovirus, and hMPV. Epidemiology, risk, and attributable mortality The impact of influenza infection in SOT patients can be particularly severe, especially in lung transplant recipients. 78 Reports of the most severe infections are largely based on outcomes following the 2009 H1N1 pandemic; however, a recent retrospective cohort study of Brazilian renal transplant recipients with influenza A between 2009 and 2014 reported a 14% incidence of both intensive care unit (ICU) admission and mortality, which is higher than expected.
cord-276952-nkaow79h	2018	Viral respiratory tract infection (vRTI) is a significant cause of morbidity and mortality in patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT). This study aimed to assess the epidemiologic characteristics, risk factors, and outcomes of vRTI occurring in the period from conditioning to 100 days after allo-HSCT in the era of molecular testing. Demographic and clinical data were collected from hospital clinical records using a case report form and included age, sex, underlying disease, previous therapy, stem cell source, conditioning therapy, graft-versus-host disease (GVHD), and outcomes (ie intensive care unit [ICU] admission and death). For patients with RV infection, the number of vRTIs, type of RVs, clinical presentation, antiviral therapy, and outcomes (ie, ICU admission, death, use of mechanical ventilation, and progression to lower respiratory tract infection [LRTI]) were also obtained during the 100-day period.
cord-279638-jr1mbh7s	2015	Acute graft-versus-host disease (aGVHD) is the major cause of morbidity and mortality after allogeneic hematopoietic stem cell transplantation. Update on the mechanism of action and on clinical efficacy of extracorporeal photopheresis in the treatment of acute and chronic graft versus host disease in children Extracorporeal photopheresis (photochemotherapy) in the treatment of acute and chronic graft versus host disease: immunological mechanisms and the results from clinical studies Role of extracorporeal photopheresis (ECP) in treatment of steroid-refractory acute graft-versus-host disease Extracorporeal Photopheresis for the treatment of acute and chronic graft-versus-host disease in adults and children: best practice recommendations from an Italian Society of Hemapheresis and Cell Manipulation (SIdEM) and Italian Group for Bone Marrow Transplantation (GITMO) consensus process Extracorporeal photopheresis for steroid resistant graft versus host disease in pediatric patients: a pilot single institution report Extracorporeal photochemotherapy in graft-versus-host disease: a longitudinal study on factors influencing the response and survival in pediatric patients
cord-280763-4bnv2t3f	2019	METHODS: In this prospective, cross-sectional study, we retrospectively analyzed the effect of inactivated trivalent influenza vaccination on the prevalence of influenza RVI in a consecutive cohort of 136 allo-HSCT adult recipients who developed 161 RVI over 5 flu seasons (from 2013 to 2018). The influenza virus has a significant impact on morbidity and mortality in allogeneic hematopoietic stem cell transplantation patients (allo-HSCT), leading to complications ranging from self-limited upper-respiratory tract infections to life-threatening or fatal pneumonias [1] [2] [3] [4] . We conducted a prospective, cross-sectional, observational epidemiological study of community-acquired respiratory virus (CARV) respiratory tract disease (RTD) in allo-HSCT recipients who developed upper RTD (URTD) and/or lower RTD (LRTD) symptoms after transplant. In this study, we report the prevalence of influenza RTD according to the vaccination status over 5 consecutive influenza seasons in a consecutive series of allo-HSCT recipients with virologically-documented respiratory virus infections (RVIs).
cord-281026-2avisuge	2010	title: Strong Association between Respiratory Viral Infection Early after Hematopoietic Stem Cell Transplantation and the Development of Life-Threatening Acute and Chronic Alloimmune Lung Syndromes Alloimmune lung syndromes (allo-LS), including idiopathic pneumonia syndrome, bronchiolitis obliterans syndrome, and bronchiolitis obliterans organizing pneumonia, are severe complications after hematopoietic stem cell transplantation (HSCT). Multivariate analysis showed that respiratory viral infection early after HSCT is an important predictor for the development of allo-LS (P <.0001). To analyze risk factors for outcomes, we considered variables associated with the recipient (age at transplantation, sex, CMV serology, RV positivity, single/multiple viruses), the disease (malignant vs nonmalignant), the donor/transplantation technique (cell source, HLA disparity, donor relationship, conditioning regimen), HSCT complications (allo-LS, acute GVHD [aGVHD], CMV and adenovirus plasma DNA positivity, venoocclusive disease), and relapse. Prospective study of respiratory viral infections in pediatric hemopoietic stem cell transplantation patients
cord-283141-dh8j7lyl	2020	[10] [11] [12] [13] [14] [15] [16] [17] [18] [19] [20] [21] [22] Since the parental consanguinity rate is high (23.2%) in Turkey, DOCK8 deficiency has an important place among CIDs. Here, we retrospectively evaluated the clinical and immunologic features and treatment modalities of 20 patients with DOCK8 deficiency and follow-up results of hematopoietic stem cell transplant (HSCT) in 11 patients among them as a single-center experience. clinic, DOCK8 deficiency, follow-up, hematopoietic stem cell transplantation, immunological features Hematopoietic stem cell transplantation outcomes for 11 patients with dedicator of cytokinesis 8 (DOCK8) deficiency Successful engraftment of donor marrow after allogeneic hematopoietic cell transplantation in autosomal-recessive hyper-IgE syndrome caused by dedicator of cytokinesis 8 deficiency Clinical and immunological correction of DOCK8 deficiency by allogeneic hematopoietic stem cell transplantation following a reduced toxicity conditioning regimen Successful hematopoietic stem cell transplantation after myeloablative conditioning in three patients with dedicator of cytokinesis 8 deficiency (DOCK8) related Hyper IgE syndrome
cord-291966-5jm5c2lj	2013	title: Outcomes of Hematopoietic Stem Cell Transplant Recipients with Rhinovirus Infection: A Matched, Case-Control Study The controls were defined as patients who underwent HSCT in the same study period, and who were never diagnosed with rhinovirus, whether they had PCR testing of the upper or lower respiratory tracts or not. 1 The following data were collected after reviewing the patients'' medical records: age, gender, ethnicity, underlying malignancy, type of HSCT, medical comorbidities, conditioning regimen, immunosuppression and anti-infective prophylaxis at the time of rhinovirus diagnosis, laboratory findings at the time of rhinovirus diagnosis ( ± 7 days), other infections after rhinovirus diagnosis, CMV and EBV infection or disease after transplant, in addition to outcomes at the end of the follow-up period including mortality, hospitalizations and GVHD. Of these, other upper and lower respiratory tract infectious diseases were the cause of recurrent hospitalizations in seven cases and six controls (P ¼ 0.125).
cord-292645-5nplyyai	2013	In the immunocompromised populations, particularly those undergoing chemotherapy for malignancy and solid organ and hematopoeitic stem cell transplant patients, viral respiratory infections can cause high rates of morbidity and mortality. Several case reports of pediatric immunocompromised hosts, including those with HSCT, leukemia, and liver transplantation, appear in the literature, predominantly with oseltamivir-resistant pandemic influenza H1N1 viral isolates. Despite limited data on its efficacy in clearing RSV infection in solid organ transplant (SOT), ribavirin, especially in the aerosolized form, has additionally been evaluated in conjunction with other therapies, including steroids, IVIG, and palivizumab (see below), and remains a standard part of many local treatment protocols. The current antiviral armamentarium against respiratory viral infections aside from influenza therapies is limited, resulting in few options to impact the associated morbidity and mortality from these illnesses in immunocompromised hosts. Respiratory failure caused by 2009 novel influenza A/H1N1 in a hematopoietic stem-cell transplant recipient: detection of extrapulmonary H1N1 RNA and use of intravenous peramivir
cord-294150-eq2vkm4i	2016	BACKGROUND AND OBJECTIVE: High‐dose chemotherapy (HDCT) followed by autologous haematopoietic stem cell transplantation (HSCT) is widely used in paediatric cancer patients, but few data about noninfectious interstitial lung disease (ILD) following this treatment are available. High-dose chemotherapy (HDCT) followed by autologous haematopoietic stem cell transplantation (HSCT) is widely used as a treatment for advanced or refractory paediatric solid tumours. Twenty-five parameters were analysed as potential risk factors for noninfectious ILD, including age at HDCT; gender; underlying tumour diagnosis; use of each chemotherapy agent (cisplatin, etoposide, cyclophosphamide, carboplatin, vincristine, ifosfamide, doxorubicin, methotrexate, actinomycin D and bleomycin); use of each HDCT agent (carboplatin, thiotepa, melphalan, cyclophosphamide, etoposide, busulfan and ifosfamide); MIBG treatment; interleukin-2 usage; number of HDCT treatments; total body irradiation; and thoracic field radiation therapy. In this study, the incidence of noninfectious ILD following HDCT and autologous HSCT in paediatric Interstitial lung disease and transplant solid tumour patients was 2.4%.
cord-294906-1m4h116m	2020	title: SARS‐CoV‐2 viral clearance during bone marrow aplasia after allogeneic hematopoietic stem cell transplantation – a case report Here, we report a unique case of a child with viral pneumonia caused by coinfection with human metapneumovirus (MPV), respiratory syncytial virus (RSV), and SARS‐CoV‐2 after HSCT. CONCLUSIONS: Posttransplant care in HSCT recipients with COVID‐19 infection is feasible in regular transplant units, provided the patient does not present with respiratory failure. Early and repeated testing for SARS‐CoV‐2 in posttransplant patients with concomitant infection mitigation strategies should be considered in children after HSCT who develop fever, respiratory symptoms and perhaps gastrointestinal symptoms to control the spread of COVID‐19 both in patients and healthcare workers in hospital environments. The epidemiology and clinical characteristics of co-infection of SARS-CoV-2 and influenza viruses in patients during COVID-19 outbreak Mortality from Respiratory Virus Infections within the First One Hundred Days in Children after Hematopoietic Stem Cell Transplantation
cord-313474-1gux1gsi	2015	Materials (or patients) and methods: We performed a multicenter, multinational, open-label, randomized study comparing anti-lymphocyte globulin (ATG-Fresenius s ) 10 mg/kg on day -3, -2 and -1 with no ATG in patients with AML (n ¼ 110) or ALL (n ¼ 45) in 1 st complete remission (CR; n ¼ 139) or 2 nd CR (n ¼ 16) who received peripheral blood stem cells from their HLA-identical sibling after standard TBI (12 Gy)/Ccclophosphamide (120 mg/kg) or busulfan (16 mg/ kg)/Cy (120 mg/kg) based myeloablative conditioning regimen. After allo-HSCT, detection of positive WT1 was followed by immunomodulatory therapeutic interventions according to the time from transplant, the presence of active graft-versus-host disease (GvHD) and the general clinical conditions: tapering and/or discontinuation of immunosuppressive drugs (IS), donor lymphocytes infusions (DLI), administration of hypomethylating agents. Introduction: Haploidentical hematopoietic stem cell transplantation(Haplo-HSCT)is feasible option for patients with acute leukemia(AL)at high risk of relapse who do not have HLA-matched related or unrelated donors.
cord-322606-cx6eh0ff	2005	Our objective was to study the outcome of allogeneic hematopoietic stem cell transplantation (HSCT) for Shwachman-Diamond Syndrome (SDS). Our objective was to study the outcome of allogeneic hematopoietic stem cell transplantation (HSCT) for Shwachman-Diamond Syndrome (SDS). Polychemotherapy with an AML-like regimen, including high-dose cytarabine, mitoxantrone, VP16 and amsacrine, was administered to one of the five patients with MDS/ leukemia; it resulted in partial disease control and disappearance of the cytogenetic clone, but cytological bone marrow abnormalities persisted (about 5% blasts). We report the outcome of HSCT in 10 patients with SDS and severe hematological complications (bone marrow failure in five cases and myelodysplasia/AL in five cases). Successful allogeneic hematopoietic stem cell transplantation (HSCT) for Shwachman-Diamond syndrome Successful unrelated donor bone marrow transplantation for Shwachman-Diamond syndrome with leukemia Allogeneic bone marrow transplantation in Shwachman-Diamond syndrome with malignant myeloid transformation. Liver failure complicating non-alcoholic steatohepatitis following allogeneic bone marrow transplantation for Shwachman-Diamond syndrome
cord-322822-z0ehfrg0	2005	Although case reports of viral pneumonia complicating hematopoietic stem cell transplantation (HSCT) or solid organ transplantation (SOT) have been described for decades, it is only in recent years that larger case series and therapeutic trials have been conducted and reported, providing greater insight into the impact of CARV on these immunosuppressed hosts. After some general observations about CARV infections, this article focuses on this important recent literature and specifically on the four most common pathogens, respiratory syncytial virus (RSV), influenza virus, parainfluenza virus (PIV), and adenovirus. Because these series do not include patients who had asymptomatic infection, overall reported rates of CARV infection are predictably lower, ranging from 4% to 27% in HSCT recipients [1 -3,11 ] to 8% to 21% in lung transplant recipients [8, 12, 13] .
cord-339931-e2ylkonb	2018	We aimed to evaluate the treatments, particularly the role of corticosteroids, in patients with late-onset hemorrhagic cystitis (LOHC) after allogeneic hematopoietic stem cell transplantation (allo-HSCT). There are several therapeutic methods for LOHC, including ensuring appropriate hydration, hematological homeostasis (maintaining high platelet counts, appropriate red cell counts, and levels of clotting factors), pain relief, catheterization for cystoscopic clot extraction, continuous bladder irrigation with normal saline for prevention of clots and bladder tamponade, anti-infection (particularly antiviral), hyperbaric oxygen, estrogen, clotting factors, and keratinocyte growth factor therapies [5] . However, for the patients having concurrent grade II to IV acute GVHD and refractory LOHC, systemic corticosteroid therapy should be added immediately with the use of empirical antiviral therapies or anti-CMV therapy (Fig. 1) . For patients showing unsatisfactory response to anti-infection therapies, additional corticosteroid therapy may help to achieve CR, particularly for those with severe LOHC. Hemorrhagic cystitis following hematopoietic stem cell transplantation: incidence, risk factors and association with CMV reactivation and graft-versushost disease
cord-347761-wgodcsav	2009	Pneumocystis jiroveci pneumonia (PCP), CMV and Aspergillus are particularly important and well recognized sources of infection in the immunocompromised host; however, other significant pathogens have more recently been identified. Lung biopsy may be particularly important in the diagnosis of fungal infection, especially when there is a negative BAL in patients with persistent signs, symptoms or chest x-ray changes. PCP has historically been associated with HIV but is also a significant cause of morbidity in other groups of immunocompromised patients, particularly those with haematological malignancies, brain tumours requiring prolonged courses of steroids, prolonged neutropaenia or lymphopaenia, and those undergoing HSCT. Prophylaxis to prevent CMV and HSV reactivation is used for children undergoing HSCT and many SOTs. Surveillance in high-risk patients enables pre-emptive treatment to be given before damaging disease occurs. Adenovirus is usually responsible for relatively minor upper respiratory tract or gastrointestinal infection but can result in life-threatening pneumonia, meningitis, encephalitis and disseminated disease in the immunocompromised.
cord-348130-t9tysvr8	2018	In addition, the types and risk factors of infectious complications differ according to the stem cell source, donor type, conditioning intensity, region, prophylaxis strategy, and comorbidities, such as graft-versushost disease and invasive fungal infection. Bacteria: consider fluoroquinolone a Fungus: consider prophylaxis during neutropenia, consider PCP prophylaxis Virus: during neutropenia or longer depending on risks HSCT, hematopoietic stem cell transplantation; PCP, Pneumocystis jirovecii pneumonia; GVHD, graft-versus-host disease; TNF-α, tumor necrosis factor-α. In a systematic review and meta-analysis of non-HIV immunocompromised hosts (patients with acute leukemia and recipients of HSCT and solid organ transplant), the incidence of PCP was reduced by 91% (relative risk [RR], 0.09) in trimethoprim/sulfamethoxazole prophylaxis group compared with placebo [69, 70] . Epidemiology and risk factors for invasive fungal diseases among allogeneic hematopoietic stem cell transplant recipients in Korea: results of