Diseases of the Blood

At the end of this course, students should be able to: 1. define haematopoietic system and describe the components of HS, blood cells, the bone marrow, and lymphoid organs; 2. illustrate the development of HS; 3. describe the evaluation of child with haematologic disorders, 4. explain epidemiology and pathogenesis; 5. enumerate the classification of thalassemia and describe the diagnosis of α- thalassaemia and β-thalassaemia; 6. list complications/late stages; 7. describe acute lymphoblastic leukaemia and acute myeloid leukaemia; 8. enumerate the classification of acute leukaemias and list the clinical features of acute leukaemia; 9. discuss principles of induction, consolidation and maintenance cytotoxic chemotherapy, including attendant side effects; and 10. explain the outcomes of leukaemia according to classification and prognostic indices.

Evaluation of haematopoietic system (HS). Definition of haematopoietic system. The components of HS (blood cells, the bone marrow, and lymphoid organs). Development of HS. Evaluation of child with haematologic disorders. Anaemia in children. General approach to anaemia. Introduction. Definition of anaemia. Patient characteristics-age of patient-sex-race and ethnicity. Evaluation-history-physical examination. Laboratory evaluation-complete blood count-haemoglobin and haematocrit-RBC indices-white blood count and platelet count-blood smear reticulocyte count. Haemoglobin electrophoresis, classification of anaemia, microcytic anaemia, normocytic anaemia, macrocytic anaemia, reticulocyte response. Confirmatory testing. Sickle cell disease and other haemoglobinopathies basic concept of SCD, definition, epidemiology. Pathogenesis/pathophysiology. Factors enhancing sickling. Haplotype. Sickle cell crises, vaso-occlusive pain episode, acute chest syndrome, stroke, hyper-haemolytic crisis, aplastic crisis, sequestration crisis, priapism, osteomyelitis, avascular necrosis, sickle cell nephropathy, sickle cell hepatopathy. Thalassemia, definition, epidemiology, pathogenesis, classification, diagnosis, α-thalassaemia, β-thalassaemia, overview clinical manifestations, early manifestations, skeletal changes, liver and gallbladder, splenomegaly, splenectomy, kidneys, management. Acute leukaemias, acute lymphoblastic leukaemia, acute myeloid leukaemia. classification of acute leukaemias integrating, morphology, cytochemistry, cytogenetics. Clinical features of acute leukaemia. Principles of induction, consolidation and maintenance cytotoxic chemotherapy, including attendant side effects. Outcomes of leukaemia according to classification, prognostic indices. Bleeding disorders, coagulation, mechanism of clotting, evaluation of abnormal coagulation tests, inherited coagulation disorders. Haemophilia a, haemophilia b, Von-Willebrand disease. Introduction. Definitions. Epidemiology, clinical manifestations, age at first bleeding, initial site of bleeding, intracranial bleeding, joints and muscle, epistaxis, oral, gastrointestinal bleeding, laboratory evaluation. Haemophilic arthropathy, development of inhibitors, diagnostic evaluation, patient and family history. Laboratory testing, screening tests, factor activity levels, genetic testing. 133