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

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Presentation on theme: "Introduction To Haematological Malignancies By Tiffany Shaw MBChB II July 2002."— Presentation transcript:

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

2 Common Malignancies AcuteChronic Lymphoid Acute lymphoblastic leukemia Chronic lymphocytic leukemia Lymphoma Myeloma Myeloid Acute myeloid leukemiaChronic myeloid leukemia

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 1. Bone marrow infiltrated by blasts cells 2. Bone marrow failure 3. Pan-cytopenia 4. Anaemia  fatigue, SOB on exersion 5. Thrombocytopenia  bleeding / DIC 6. Neutropenia  infections 7. Constitutional symptoms (malaise, anorexia, night sweat, fever) 8. Lymphadenopathy, hepatospenomegaly

5 Acute Leukemias -- Ix 1. Low Hb 2. Low platelets 3. High WCC (increased blast cells) 4. Low neutrophils 5. Elevated serum uric acid 6. Bone marrow biopsy: infiltration by blast cells

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

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

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

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

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

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: StageABC < 3 lymph node groups involvement Bloods normal > 3 lymph node groups involvement Bloods normal Bloods abnormal (anaemia + thrombocyto penia)

23 CLL -- Mx 1. Observation (stage A) 2. Chlorambucil PO (lower lymphocyte count and lymphadenopathy) 3. Corticosteroid 4. Fludarabine IV 5. Other chemo Rx 6. 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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