key: cord-0724192-xy74wd6j
authors: Sora, Federica; Chiusolo, Patrizia; Laurenti, Luca; Innocenti, Idanna; Autore, Francesco; Alma, Eleonora; Viscovo, Marcello; Fusco, Domernico; Maresca, Maddalena; Tumbarello, Mario; Sica, Simona
title: SARS COV 2 INFECTION IN CHRONIC MYELOGENOUS LEUKEMIA: SEVERE HEMATOLOGICAL PRESENTATION
date: 2020-07-23
journal: Transfus Apher Sci
DOI: 10.1016/j.transci.2020.102881
sha: 262085135d9c9d8350b290d9183c64526798b374
doc_id: 724192
cord_uid: xy74wd6j

Abstract Infection with SARS-CoV-2, the cause of coronavirus infectious disease-19 (COVID-19), has caused a pandemic. Few data are available about the risk of COVID-19 infection in persons with hematological cancer, but controversy whether these persons have the same clinical signs and outcomes. We describe a case of life‐threatening COVID-19 infection complicated by severe anemia in patients affected also by chronic myelogenous leukemia. The screening for RBC antibodies and the direct antiglobulin test (DAT) turned positive. The identification of the antibodies, showed the presence of an alloantibody with anti-Lewis b specificity, which was reactive at room temperature, in the anti-human globulin phase (AGH) and with papain-treated red blood cells. At the same time hemophagocytic lymphohistiocytosis (HLH), on the basis of major laboratory findings including hyperferritnemia, increase of triglicerides levels and according to the HLH score was suspected. Patients received antiviral therapy, steroids and intravenous immunoglobulins. Hemolysis resolved and ferritin dramatically decreased after administration of Ig and a Afull recovery was achieved after viral infection resolution.This case highlights the novel and multifaceted hematological findings during sever COVID 19 infection. COVID 19-related pneumonia is mediated by hyper activation of effector T cells and excessive production of inflammatory cytokines, such as IL-6, IL-1, interferon-gamma, and TNF. This inflammatory process called "cytokine storm" is a life-threatening complication of COVID 19 infection. In this case severe immunohematological consequences are reported for the first time and recognition of this complications are probably underestimated.

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Federica Sora 1,2* , Patrizia Chiusolo 1,2* , Luca Laurenti 1, 2 

Infection with SARS-CoV-2, the cause of coronavirus infectious disease-19 (COVID-19), has caused a pandemic .Few data are available about the risk of COVID-19 infection in persons with hematological cancer, but controversy whether these persons have the same clinical signs and outcomes. We describe a case of life-threatening COVID-19 infection complicated by severe anemia in patients affected also by chronic myelogenous leukemia . The screening for RBC antibodies and the direct antiglobulin test (DAT) turned positive. The identification of the antibodies, showed the presence of an alloantibody with anti-Lewis b specificity, which was reactive at room temperature, in the anti-human globulin phase (AGH) and with papain-treated red blood cells. At the same time hemophagocytic lymphohistiocytosis (HLH), on the basis of major laboratory findings including hyperferritnemia, increase of triglicerides levels and according to the HLH score was suspected. Patients received antiviral therapy, steroids and intravenous immunoglobulins. Hemolysis resolved and ferritin dramatically decreased after administration of Ig and a Afull recovery was achieved after viral infection resolution.This case highlights the novel and multifaceted hematological findings during sever COVID 19 infection. COVID 19related pneumonia is mediated by hyper activation of effector T cells and excessive production of inflammatory cytokines, such as IL-6, IL-1, interferon-gamma, and TNF. This inflammatory process called "cytokine storm" is a life-threatening complication of COVID 19 infection. In this case severe immunohematological consequences are reported for the first time and recognition of this complications are probably underestimated. immunoglobulins (IVIG) 20 gr/day were administered for 2 consecutive days. The patient received seven RBC units compatible tested at 37°C, infused using in-line blood warmer without presenting acute or late hemolytic reactions. Four weeks later , antibody screening and identification performed at room temperature, in AGH and with enzyme-treated red cell (Identisera and Identisera P, Grifols, Spain), were completely negative. DAT turned weakly positive (score of agglutination 0.5 +) and only for IgG component (DC Screening, Bio-Rad, Switzerland). Only cold agglutinins at very low title and reactive just at 4°C were detected. Reticulocytes crisis was observed shortly after IVIG. In this particular case several mechanism seem to be elicited from SARS-COV2 infection giving origin to multifaceted hematological findings. Potential immune-mediated injury in sever COVID 19 seems to be quite frequent and appropriate immunosuppressive treatment particularly in prone subjects can be considered.COVID 19-related pneumonia is mediated by hyper activation of effector T cells and excessive production of inflammatory cytokines, such as IL-6, IL-1, interferon-gamma, and TNF. This inflammatory process may cause a pathological process that leads to plasma leakage, vascular permeability, and disseminated intravascular coagulation; this reaction, called "cytokine storm" is a life-threatening complication of COVID 19 infection. In this case severe immunohematological consequences during SARS-COV-2 are reported and recognition of this complications are probably underestimated, as a matter of fact in May 2020, 9 cases of AIHA and SARS-COV2 infection were published (3) (4) (5) and only 3 of them showed cold antibodies mainly in the context of a lymphoproliferative disorder (6).

Considering the unusual frequency of chilblainses in children with SARS-COV2 infectionit will be interesting to study the presence of cold agglutinins and cryoproteins in this particular setting to address if SARS-COV2 may be included in the list of viral agents causing cold agglutinins.

COVID-19: consider cytokine storm syndromes and immunosuppression

Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome

Evans syndrome in a patient with COVID-19

Simultaneous onset of COVID-19 and autoimmune haemolytic anaemia

Autoimmune hemolytic anemia associated with Covid-19 infection