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Introduction To Haematological Malignancies

Introduction To Haematological Malignancies. By Tiffany Shaw MBChB II July 2002. Common Malignancies. Acute Leukemias -- Definition. A malignant disorder in which haemopoietic blast cells proliferate and constitute > 30% of the bone marrow. Lymphoblasts  Lymphoblastic leukemia

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Introduction To Haematological Malignancies

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  1. Introduction To Haematological Malignancies By Tiffany Shaw MBChB II July 2002

  2. Common Malignancies

  3. Acute Leukemias -- Definition A malignant disorder in which haemopoietic blast cells proliferate and constitute > 30% of the bone marrow. Lymphoblasts  Lymphoblastic leukemia Myeloblasts  Myeloid leukemia

  4. Acute Leukemias -- PC • Bone marrow infiltrated by blasts cells • Bone marrow failure • Pan-cytopenia • Anaemia  fatigue, SOB on exersion • Thrombocytopenia  bleeding / DIC • Neutropenia  infections • Constitutional symptoms (malaise, anorexia, night sweat, fever) • Lymphadenopathy, hepatospenomegaly

  5. Acute Leukemias -- Ix • Low Hb • Low platelets • High WCC (increased blast cells) • Low neutrophils • Elevated serum uric acid • Bone marrow biopsy: infiltration by blast cells

  6. Acute Leukemias – Risk Factors • Familial • Down’s syndrome • Viral infection (EBV  Burkitt’s) • Radiation • Chemotherapy

  7. AML Myeloblastic Any age 1/3 transformation from other myeloproliferative disorders Increased DIC ALL Lymphoblastic Commonly children Primary event More lymphadenopathy and hepatosplenomegaly Acute Leukemias – AML VS ALL

  8. Acute Leukemias -- Classification French-American-British Classification • Morphology • Cytochemistry • Immunophenotype • Cytogenetics • DNA analysis

  9. AML Promyelocytic Myelomonocytic Monocytic Erythroid Megakeryoblastic Undifferentiated …. ect. ALL Small cells Large cells Vacuolated basophillic blast cells Examples

  10. Acute Leukemias -- Mx Supportive Rx • Anaemia: blood transfusion prn • Bleeding: platelets prn • Infection: antibiotics prn • Social support + issues

  11. Acute Leukemias -- Mx Chemotherapy: • Remission-induction phase • Consolidation phase • Maintenance phase (for ALL) prednisone, daunorubicin, vincristine, thioguanine, cyclophosphamide…

  12. Acute Leukemias -- Mx Bone Marrow Transplantation: • High-dose chemo Rx • Removal of stem cells from donor • Transplantation into recipient

  13. AML ~ 30-40% cured Varies widely with age, co-morbidity..etc. ALL In children, ~ 70% cured In adults, ~ 30-40% Acute Leukemias -- Prognosis

  14. Chronic Myeloid Leukemia • Increase in neutrophils and their precursors • Really a myeloproliferative disorder • > 95% patients have chromosomal mutation --- Philadelphia chromosome

  15. CML -- PC • Constitutional symptoms (anorexia, weight loss, fatigue, night sweat) • Splenomegaly (hypochondrial pain) • Asymptomatic – incidental finding

  16. CML -- Ix • Increased WCC (neutrophils and precursors) • Later  bone marrow failure (anaemia + thrombocytopenia) • Raised serum B12 • Raised serum uric acid • Bone marrow biopsy  hypercellular with high myeloid:erythroid ratio

  17. CML – Course/Progression Chronic Phase • Raised WCC • Constitutional symptoms / Asymptomatic Accelerated Phase • Anaemia + thrombocytopenia • Splenomegaly • Bone marrow fibrosis Acute Phase • Transformation to acute leukemia (80% AML, 20% ALL)

  18. CML -- Mx Chronic Phase • Hydroxyurea • Interferons • Chemo Rx • Bone marrow transplantation Acute Phase • Same as acute leukemia

  19. Chronic Lymphcytic Leukemia • Increased lymphocytes (B cells) in the blood, bone marrow, lymph nodes, and spleen. • Lymphoproliferative disorder

  20. CLL -- PC • Asymptomatic • Lymphadenopathy • Constitutional symptoms (night sweat, weight loss, anorexia) • Symptoms of bone marrow failure • Splenomagaly

  21. CLL -- Ix • Lymphocytosis (B cells) • Low serum Ig • Raised serum uric acid • Bone marrow biopsy  lymphocytic infiltration

  22. CLL -- Ix • Depends on stage:

  23. CLL -- Mx • Observation (stage A) • Chlorambucil PO (lower lymphocyte count and lymphadenopathy) • Corticosteroid • Fludarabine IV • Other chemo Rx • Supportive care

  24. CLL -- Prognosis • Variable • May remain stationary or progress • Some stay asymptomatic for > 10 years • Cause of death = bone marrow failure, infiltration of other organs

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