key: cord-0046396-b8t42a54
authors: Bryson, Staci; Mulne, Arlynn F.
title: Blood and Neoplastic Disorders
date: 2015-03-28
journal: Pediatric Board Study Guide
DOI: 10.1007/978-3-319-10115-6_16
sha: 2e5595db345da5ac9058305a09bdf4bc1b9b0fa3
doc_id: 46396
cord_uid: b8t42a54

Blood Disorders covers disorders of red blood cells, white blood cells, platelets, and coagulation including congenital and acquired disorders. Neoplastic Disorders covers both Hematopoietic and Solid Tumors with information on epidemiology, clinical presentation, diagnosis, and treatment of these disorders.

-Downsides; taste, Gl irritability, and constipation (more water and fiber can solve this problem). • Rapid correction of anemia with transfusion may precipitate heart failure. • In severely anemic children (< 4 gm/dl) transfusions can be administered at a very slow rate (2-3 ml/kg). • If there is evidence of heart failure present, a modified exchange transfusion using fresh PRBCs can be considered. • Changes after treatment with iron.

− Within 12-24 h: irritability decreases, increased appetite. − 36-48 h: initial bone marrow response with erythroid hyperplasia. − 48-72 h: reticulocytosis, peaking at 5-7 days. − 1-3 months: repletion of stores. • Hgb may increase by 0.5 g/dl/day. • Iron therapy should be continued for at least 2 months after the Hgb normalizes to replenish iron stores. • Limit cow's milk to less than 500 cc/day. 

• VWF is a carrier protein for factor VIII • VWF stored in platelets and endothelial cell • VWF adheres to exposed the subendothelial matrix after vascular damage causing platelets to adhere via glycoprotein IB receptors on the VWF

• VWD usually have symptoms of mucocutaneous hemorrhage • Excessive bruising, epistaxis, menorrhagia, post-operative bleeding (e.g., tonsillectomy, wisdom teeth extraction) • Females more commonly diagnosed than males secondary to menorrhagia -Any menstruating female with iron deficiency, should have a detailed history of bruising and other bleeding symptoms -Stress doubles or triples level of VWF

• No single assay to rule out or diagnose VWF -Bleeding time or PFA -PTT-often prolonged but frequently normal in type 1 VWD -VWF antigen -VWF Ristocetin cofactor activity -Plasma factor VIII activity -VWF multimers -Platelet count 

• ALL: Peripheral blood usually shows leukocytosis with a population of large mononuclear cells (Fig. 12) • AML: Peripheral blood usually shows myeloblasts with a high ratio of nucleus to cytoplasm (Fig. 13) 

• Per local or national protocols Associated syndromes/risk factors • ALL -Down's syndrome. • Acute leukemia is 34 times more common in children with Down's syndrome. • 20-30 % will develop leukemia by age 3 years.

• Ratio of ALL and AML is the same as the general population. • AML has a better outcomes in children with Down's syndrome. • 10 % of neonates with Down's syndrome may develop a transient leukemia or myelodysplastic syndrome. ▪ Characterized by a high leukocyte count, blast cells, anemia, thrombocytopenia and hepatosplenomegaly. ▪ Resolve within days to weeks from initial presentation. -Ataxia-telangiectasia. -Bloom's syndrome.

• Immunodeficiency, progeria, growth retardation.

• Chromosome fragility/breakage. (Fig. 17) • 40 % of all CNS tumors • Juvenile pilocytic astrocytoma-most common subtype in children • Classic site for JPA is cerebellum, but can occur anywhere in CNS -Treatment

• Surgery-primary treatment • Chemotherapy • Radiation therapy -Medulloblastoma (Fig. 16) • 20 % of all brain tumors (second most common) Wilms Tumor (Fig. 19) • WT-1 gene located on 11p13 

Neutrophil abnormalities

Congenital neutropenia: diagnosis, molecular bases and patient management

Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial

When is lymph node biopsy indicated in children with enlarged peripheral nodes?

Priapism in sickle cell disease