Pediatric Hematology Important Topics 

BASICS OF HEMATOPOIESIS

• Hematopoiesis
• Three stages of Hematopoiesis
• Sequence of differentiation of Hematopoietic Cells
• Fetal Granulocytopoiesis
• Fetal Thrombopoiesis
• Fetal Erythropoiesis
• Structure of adult hemoglobin
• Hemoglobins in the fetus and neonates
• Two major switches in the beta-globin gene
• Alterations of hemoglobin in diseases

ANEMIAS IN CHILDREN

• Definition of Anemia
• WHO cut-offs of HB for anemia to remember
• Physiological Adjustments in Anemia
• Clinical features of Anemia
• Abnormal Findings in Anemia
• Clues for Specific Diagnosis and Etiology
• Peripheral Findings in Anemia
• Investigations for Anemia
• Revised WHO Cut-offs for Anemia

HEMOLYTIC ANEMIAS

• Hemolysis & Hemolytic anemia
• Clinical Features Suggestive of Hemolysis
• Features Commonly Encountered in Hemolytic Anemia
• Classification of Hemolytic Anemia
• Problems with Hemolysis
• Intravascular and Extravascular Hemolysis
• Diagnostic Clues based on RBC Shapes
• Hereditary Spherocytosis (HS)
• Genes and Protein Implications
• Classification of Severity
• Clinical Features
• Investigations
• Management
• Hereditary Elliptocytosis and Related Disorders
• Investigations and Management

SICKLE CELL DISEASE

• Pathophysiology
• Genetic Mutation/Haplotypes
• Spectrum of Sickle cell disease
• Crisis and Complications
• Management of SCD
• Visual spotter-Sickle Cell Disease on PBF
• New drugs in Sickle Cell Anemia
• Gene Therapy in SCA
• New Drug in β - Thalassemia
• Gene Therapy in β- Thalassemia

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

• Paroxysmal Nocturnal Hemoglobinuria
• GPI-Anchored Protein Found Deficient in PNH
• Clinical features of PNH
• Classification of PNH
• Investigations in PNH
• Treatment of PNH
• Management of PNH based on disease classification
• CATCH criteria in PNH

POLYCYTHEMIA IN CHILDREN

• Neonatal Polycythemia
• Relationship between hematocrit and viscosity
• Epidemiology of Neonatal Polycythemia
• Clinical features of Neonatal Polycythemia
• Screening Protocol
• PET- Partial Exchange Transfusion
• Peripheral versus umbilical route for PET
• Partial exchange transfusion for polycythemia
• Types of Polycythemia in Children
• Major causes of Polycythemia
• Approach to polycythemia
• Polycythemia Vera (PV)
• Clinical Features and Management
• Management
• New drug in PV
• Fact to remember

PANCYTOPENIA AND INHERITED BMFs

• What is Pancytopenia
• Inherited Bone Marrow Failure Syndromes (IBMFSs)
• Fanconi Anemia
• Management of FA
• Shwachman-Diamond Syndrome (SDS)
• Dyskeratosis Congenita (DC)
• Congenital Amegakaryocytic Thrombocytopenia (CAMT)
• Dubowitz Syndrome
• Seckel Syndrome
• Syndrome / Entities where Pancytopenia may sometimes occur
• Acquired Aplastic Anemia

BLEEDING DISORDERS IN CHILDREN

• Clinical Clues: Platelet or Clotting Factor Bleed?
• Platelets
• Individual Platelet Disorders
• Chronic ITP
• TTP
• Congenital Thromobocytopenic Syndrome
• Neonatal Thrombocytopenia
• Neonatal Alloimmune Thrombocytopenia

COAGULATION DISORDERS IN CHILDREN

• Coagulation Cascade
• Intrinsic Pathway
• Extrinsic Pathway
• Common Pathway
• Coagulation Factors
• Natural Anticoagulant Proteins
• Coagulation Tests
• Tests and Possible Diagnosis
• Hemophilia A and B
• Severity of Hemophilia
• Categories of Hemophilias based on Severity
• Chronic Complications in Hemophilia
• Haemophilia C
• Difference of Hemophilia C from Hemophilia B and Hemophilia A
• Parahemophilia
• Factor XIII deficiency
• Non-Factor Therapies in Hemophilias

VON WILLEBRAND DISEASE

• Physiological Functions of Won Willebrand Factor
• Classification of VWD Disease
• Preparations of Von Willebrand factor
• Supportive Treatment

BLOOD COMPONENTS TRANSFUSION IN CHILDREN

• Components of whole blood
• A. Whole Blood
• B. RBC Transfusions
• Difference between PRBC and Leuko-reduced RBCs
• Methods of Leukodepletion
• Guidelines for Pediatric RBC Transfusions
• Additional Indications for PRBC Transfusions
• Controversial areas and scenarios discussed in Nelson 21st Edition
• C. Neutrophil or Granulocyte Transfusions (GTX)
• GTX Preparation
• Dosing of GTX
• Platelets Transfusion
• Concept of corrected count increment
• Plasma Transfusion
• Plasma Transfusion Indications

GRAFT VERSUS HOST DISEASE

• Common Organ Systems Involved Initially (Initial Target Organ)
• Staging and Grading
• Prophylaxis of Acute GVHD
• Management of Acute GVHD
• MSCs in Acute GVHD
• Chronic GVHD
• Risk Factors for Chronic GVHD
• Pathogenesis of Chronic GVHD
• Clinical Features of Chronic GVHD
• Management of Chronic GVHD

PORPHYRIAS IN CHILDREN

• Heme Biosynthesis
• Types of Porphyrias
• Acute Intermittent Porphyria
• Pathogenesis of AIP
• Factors which can Precipitate Acute Attack
• Drugs unsafe in Porphyria
• Clinical Features of AIP
• Congenital Erythropoietic Porphyria (CEP)
• Cryoprecipitate

HSCT  (Hematopoietic stem cell transplantation) IN CHILDREN

• Source of Stem Cells
• Autologous HSCT in Children
• Tandem Transplants
• Allogenic HSCT
• HLA Matching
• Donors for Allogeneic HSCT
• GVL (Graft Vs Leukemia) Effect
• HLA-Matched Unrelated Donor Transplantation
• Umbilical Cord Blood Transplantation
• Haploidentical HSCT

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