HAEMATOLOGICAL MALIGNANCY

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1 HAEMATOLOGICAL MALIGNANCY Reference Compulsory reading Haematology at Glance 2 nd ed. Atul Mehta & Victor Hoffbrand Chapters: 20 to 31 Pages: 46 to 69

2 Pathogenesis of Haematological Malignancy Figure (a) page 46 Haematology at a glance Predisposing factors / Causes of haematological malignancies Genetic predisposition eg. Down s syndrome Viral infection eg. HTLV-1, EBV, HIV Ionizing radiation Toxins / Chemicals eg. benzene Drugs eg. Melphalan

3 Classification of Haematological Malignancies Table 20.1 page 47 Haematology at a glance Acute leukaemia - Introduction Malignant disorder Haemopoeitic blast cells comprise >20% of bone marrow cells Two main groups : Acute lymphoblastic leukaemia (ALL) and acute myeloid leukaemia (AML) Both ALL and AML are sub-classified using morphological, immunological, cytochemical and cytogenetic criteria

4 Acute leukaemia Clinical manifestations Symptoms due to : Bone marrow failure Tissue infiltration Constitutional symptoms Leucostasis Other (DIC) Usually short duration of symptoms Lymphadenopathy and hepatosplenomegaly are frequent findings, especially in ALL Acute leukaemia Laboratory features Anaemia, thrombocytopenia and often neutropenia Leucocytosis caused by blast cells Bone marrow shows infitration by blast cells (>20% of all marrow cells) May have an abnormal coagulation profile Serum uric acid and lactate dehydrogenase maybe raised

5 Acute leukaemia Accumulation of blasts in the bone marrow Classification of Acute leukaemia The following tests are essential for diagnostic and prognostic information: Light microscopy morphological analysis Cytochemical staining Immunophenotyping Cytogenetics

6 Light microscopy Cytochemistry

7 Acute leukaemia treatment (1) Choice of treatment is influenced by: Type of leukaemia (AML vs ALL) Age Curative vs palliative intent Acute leukaemia treatment (2) Principles of treatment Combination chemotherapy First goal is complete remission induction, intensification/consolidation Second goal to maintain remission/prevent relapse maintenance therapy Supportive medical care Transfusions, antibiotics, nutrition Psychosocial support Stem cell transplantation

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15 Lymphoma Lymphomas (1) Lymphomas are a clonal neoplastic proliferation of lymphoid cells originating in lymph nodes or other lymphoid tissue Divided into Hodgkin s lymphoma (HL) and non- Hodgkin s lymphoma (HL) Possible co-factors/predisposing factors include: Epstein Barr virus (in HL, Burkitt s lymphoma), HTLV-1 in adult T-cell leukaemia/lymphoma, chronic Helicobacter pylori infection in gastric lymphoma, autoimmune diseases (eg. rheumatoid arthritis), immune suppression (eg. AIDS, post transplant).

16 Lymphoma (2) Painless lymphadenopathy is the characteristic presentation Definitive diagnosis is made on lymph node biospy (histological!) and NOT on peripheral blood smear or bone marrow Reed Sternberg cells are characteristic of Hodgkin s lymphoma Histological classification has prognostic significance Staging is important an it influences both treatment and prognosis Treatment depends principally on stage and includes radiotherapy, chemotherapy or combined modality therapy (radiotherapy and chemotherapy) Lymphoma (3) Presence of the Reed-Sternberg cell and its variants distinguishes HL from NHL

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