Pediatric Hematology (HEME-301) | 100 Exam Questions on Bleeding Disorders, Hemophilia & Pediatric Leukemia

This document contains 100 concise and clinically relevant exam questions with answers from the Pediatric Hematology section of the HEME-301 course at the University of Health Sciences, developed for the 2025/2026 academic year. It is structured to help students master pediatric bleeding disorders, congenital platelet defects, leukemia subtypes, and bone marrow failure syndromes with a focus on clinical presentations, diagnostic workups, and molecular correlations. Core Topics Covered: Bleeding Disorders & Coagulation Factor Deficiencies: Mixing study interpretation: • Correction → factor deficiency • No correction → factor inhibitor Hemophilia (A/B): • Clinical stratification into mild (5–30%), moderate (1–5%), and severe (<1%) based on factor activity • Typical features: hemarthrosis, cephalohematoma, delayed joint/muscle bleeding von Willebrand Disease (vWD): • Defect in primary hemostasis → mucosal bleeding, superficial bleeding • Abnormal ristocetin cofactor assay (vWD or Bernard-Soulier) • Contrast with factor deficiency disorders causing delayed, deep bleeding Thrombotic Microangiopathies & Platelet Disorders: TTP: FAT RN mnemonic — Fever, Anemia, Thrombocytopenia, Renal failure, Neurological signs; ADAMTS13 deficiency ITP: mucosal bleeds, petechiae Inherited syndromes: • TAR syndrome (RMB8A mutation) • CAMT (c-Mpl mutation) • Wiskott-Aldrich (eczema, immunodeficiency, thrombocytopenia; X-linked) • Bernard-Soulier Syndrome (defective GPIb on platelets) Pediatric Leukemia & Lymphoma: Acute leukemias: 80% ALL, 20% AML in children Of ALL: 80% B-cell, 20% T-cell, mediastinal mass in T-ALL or Hodgkin’s lymphoma Chemotherapy agents: Rituximab (anti-CD20), Brentuximab (anti-CD30), associated side effects: marrow suppression, nausea, alopecia Sickle Cell Disease & Aplasia Syndromes: Sickle cell crisis: autosomal recessive, RBC lifespan <20 days, most fatal complication = acute chest syndrome Vulnerability to encapsulated organisms: S. pneumoniae, H. influenzae, N. meningitidis Management includes hydroxyurea, bone marrow transplant Parvovirus B19 → red cell aplasia This material is ideal for: Medical students in pediatric hematology, pathology, or clinical rotations Candidates preparing for USMLE, PLAB, COMLEX, AMC, and pediatric OSCEs Pediatric nursing, pharmacy, and PA students needing a high-yield bleeding disorder review Its Q&A format supports rapid memorization and clinically oriented learning, suitable for independent study or exam group prep. Keywords: hemophilia, von Willebrand disease, ristocetin assay, factor deficiency, factor inhibitor, TTP, ITP, ADAMTS13, TAR syndrome, CAMT, Wiskott-Aldrich, Bernard-Soulier, leukemia, ALL, AML, B-cell, T-cell, sickle cell, parvovirus, acute chest syndrome, CD20, CD30, rituximab, brentuximab, platelet disorders, pediatric hematology