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Introduction To Haematological Malignancies
By Tiffany Shaw MBChB II July 2002
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Common Malignancies Acute Chronic Lymphoid Myeloid
Acute lymphoblastic leukemia Chronic lymphocytic leukemia Lymphoma Myeloma Myeloid Acute myeloid leukemia Chronic myeloid leukemia
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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
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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
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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
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Acute Leukemias – Risk Factors
Familial Down’s syndrome Viral infection (EBV Burkitt’s) Radiation Chemotherapy
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Acute Leukemias – AML VS ALL
Myeloblastic Any age 1/3 transformation from other myeloproliferative disorders Increased DIC ALL Lymphoblastic Commonly children Primary event More lymphadenopathy and hepatosplenomegaly
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Acute Leukemias -- Classification
French-American-British Classification Morphology Cytochemistry Immunophenotype Cytogenetics DNA analysis
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Examples AML Promyelocytic Myelomonocytic Monocytic Erythroid
Megakeryoblastic Undifferentiated …. ect. ALL Small cells Large cells Vacuolated basophillic blast cells
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Acute Leukemias -- Mx Supportive Rx Anaemia: blood transfusion prn
Bleeding: platelets prn Infection: antibiotics prn Social support + issues
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Acute Leukemias -- Mx Chemotherapy: Remission-induction phase
Consolidation phase Maintenance phase (for ALL) prednisone, daunorubicin, vincristine, thioguanine, cyclophosphamide…
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Acute Leukemias -- Mx Bone Marrow Transplantation: High-dose chemo Rx
Removal of stem cells from donor Transplantation into recipient
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Acute Leukemias -- Prognosis
AML ~ 30-40% cured Varies widely with age, co-morbidity..etc. ALL In children, ~ 70% cured In adults, ~ 30-40%
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Chronic Myeloid Leukemia
Increase in neutrophils and their precursors Really a myeloproliferative disorder > 95% patients have chromosomal mutation --- Philadelphia chromosome
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CML -- PC Constitutional symptoms (anorexia, weight loss, fatigue, night sweat) Splenomegaly (hypochondrial pain) Asymptomatic – incidental finding
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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
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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)
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CML -- Mx Chronic Phase Acute Phase Hydroxyurea Interferons Chemo Rx
Bone marrow transplantation Acute Phase Same as acute leukemia
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Chronic Lymphcytic Leukemia
Increased lymphocytes (B cells) in the blood, bone marrow, lymph nodes, and spleen. Lymphoproliferative disorder
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CLL -- PC Asymptomatic Lymphadenopathy
Constitutional symptoms (night sweat, weight loss, anorexia) Symptoms of bone marrow failure Splenomagaly
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CLL -- Ix Lymphocytosis (B cells) Low serum Ig Raised serum uric acid
Bone marrow biopsy lymphocytic infiltration
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CLL -- Ix Depends on stage: Stage A B C
< 3 lymph node groups involvement Bloods normal > 3 lymph node groups involvement Bloods abnormal (anaemia + thrombocytopenia)
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CLL -- Mx Observation (stage A)
Chlorambucil PO (lower lymphocyte count and lymphadenopathy) Corticosteroid Fludarabine IV Other chemo Rx Supportive care
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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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