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WHO Classification Chronic Myeloproliferative Disease

MYELOPROLIFERATIVE DISEASES CHRONIC MYELOGENOUS LEUKAEMIA By DR. FATIMA AL-QAHTANI CONSULTANT HAEMATOLOGIST. WHO Classification Chronic Myeloproliferative Disease ---------------------------------------------------------------------------------------------------------------------------

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WHO Classification Chronic Myeloproliferative Disease

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  1. MYELOPROLIFERATIVE DISEASESCHRONIC MYELOGENOUS LEUKAEMIA ByDR. FATIMA AL-QAHTANICONSULTANT HAEMATOLOGIST

  2. WHO Classification Chronic Myeloproliferative Disease --------------------------------------------------------------------------------------------------------------------------- • Chronic Myelogenous Leukaemia [Ph chromosome, t(9;22)(q34;q11), BCR/ABL- positive ] • Chronic Neutrophilic Leukaemia • Chronic Eosinophilic Leukaemia (and the hypereosinophilic syndrome) • Polycythaemia Vera • Chronic Idiopathic Myelofibrosis (with extramedullary haematopoiesis) • Essential Thrombocythaemia • Chronic Myeloproliferative Disease, Unclassifiable

  3. WHO Classification Myelodysplastic / Myeloproliferative Diseases ----------------------------------------------------------------- • Chronic Myelomonocytic Leukaemia • Atypical Chronic Myeloid Leukaemia • Juvenile Myelomonocytic Leukaemia • Myelodysplastic/Myeloproliferative Disease, Unclassifiable

  4. Myeloid DisordersUsual Features at Diagnosis

  5. Myeloproliferative DiseaseRecurring Genetic Abnormalities and Their Frequency (%)at diagnosis

  6. Chronic Myelogenous Leukaemia Presenting Manifestations Common Anaemia Splenomegaly Less Common Symptoms due to the raised metabolic rate Haemorrhagic Manifestations, especially bruising

  7. Chronic Myelogenous Leukaemia Presenting Manifestations (Cont…) Occasional • Acute abdominal pain • Bone or joint pains • Menstrual disturbances • Neurological symptoms • Priapism • Gout • Skin disorder • Disturbances of vision or hearing • Accidental discovery on routine blood examination

  8. Chronic Myelogenous Leukaemia Evolution of the disease Chronic Phase Accelerated Phase Blastic Transformation (AML) (ALL)

  9. Chronic Myelogenous Leukaemia Laboratory Investigations • CBC • Neutrophil Alkaline Phosphatase • Bone Marrow Examination. • Serum B12 & B12 binding capacity • Cytogenetic Studies (Ph1) chromosome [ t ( 9 : 22 ) ] • DNA restriction enzyme analysis BCR-ABL (Breakpoint Cluster Region)

  10. CML

  11. Chronic Myelogenous LeukaemiaAccelerated Phase • Blasts 10% to 19% of peripheral blood white cells or bone marrow cells • Peripheral blood basophiles at least 20% • Persistent thrombocytopenia (<100X109/L) unrelated to therapy or persistent thrombocytosis (> 1000X109L) unresponsive to therapy • Increasing spleen size and increase WBC count unresponsive to therapy

  12. Chronic Myelogenous LeukaemiaAccelerated Phase (Cont…) • Cytogenetic evidence of clonal evolution (i.e., the appearance of an additional genetic abnormality that was not present in the initial specimen at the time of diagnosis of chronic phase CML) • Megakaryocytic proliferation in sizable sheets and clusters, associated with marked reticulin or collagen fibrosis, and/or severe granulocytic dysplasia, should be considered as suggestive of CML-AP These findings have not yet been analyzed in large clinical studies, however, so it is not clear if they are independent criteria for accelerated phase. They often occur simultaneously with one or more of the other features listed

  13. Chronic Myelogenous LeukaemiaBlastic Phase (BP) Diagnosis is established if one or more of following is present: • Blasts 20% or more of peripheral blood white cells or bone marrow cells • Extramedullary blast proliferation • Large foci or clusters of blasts in bone marrow biopsy

  14. FAP Proposal Guidelines for distinguishing Chronic Granulocytic (CGL), atypical Chronic Myeloid (aCML) and Chronic Myelomonocytic Leukaemia (CMML)

  15. Chronic Myelogenous LeukaemiaChronic Phase (BP) Treatment • Hydroxyurea (HU): WBC/ulHU (mg/kg BW/DAY) >50,000 50 15-50,000 30 – 40 <15,000 25 • Busulphan • Alpha – Interferon: 5 MU/DAY 7.5 MU/DAY if WBC > 10,000/mcl 10 MU/DAY if WBC > 20,000/mcl 3 MU/DAY if cytopenia develops • BMT, ABMT & STEM CELL HARVEST • Gleevec

  16. Diagnosis of chronic eosinophilic leukaemia and hypereosinophilic syndrome. • Required: Persistent eosinophilia ≥ 1.5X109/L in blood, increased numbers of bone marrow eosinophils, and myeloblasts <20% in blood or marrow. • Exclude all causes of reactive eosinophilia secondary to: Allergy Parasitic disease Infectious disease Pulmonary diseases (hypersensitivity pneumonitis, Loeffler’s, etc.). collagen vascular diseases.

  17. Exclude all neoplastic disorders with secondary, reactive eosinophilia: T cell lymphomas, including mycosis fungoides, Sezary syndrome. Hodgkin lymphoma. Acute lymphoblastic leukaemia/lymphoma Mastocytosis. • Exclude other neoplastic disorders in which eosinophils are part of the neoplastic clone: chronic myelogenous leukaemia (Ph chromosome or BCR/ABD fusion gene positive). Acute myeloid leukaemia, including those with inv(16), t(16;16) (p13;q22). Other myeloproliferative disease (PV,ET,CIMF) Myelodysplastic syndromes.

  18. 4. Exclude T cell population with aberrant phenotype and abnormal cytokine production. • If there is no demonstrable disease that could cause the eosinophilia, no abnormal T-cell population, and no evidence of a clonal myeloid disorder, diagnose HES. • If all of the requirements, including conditions 1-4, have been met, and if the myeloid cells demonstrate a clonal chromosomal abnormality or are shown to be clonal by other means, or if blast cells are present in the peripheral blood (>2%) or are increased in the bone marrow (>5% but less than 19% of nucleated bone marrow cells), diagnose CEL.

  19. Diagnostic criteria for chronic neutrophhilic leukaemia. • Peripheral blood leukocytosis ≥ 25X109/L. Segmented neutrophils and bands >80% of white blood cells. Immature granulocytes (promyelocytes, myelocytes, metamyelocytes) <10% of white blood cells. Myeloblasts <1% of white blood cells. • Hypercellular bone marrow biopsy. Neutrophilic granulocytes increased in percentage and number. Myeloblasts <5% of nucleated marrow cells. Neutrophilic maturation pattern normal.

  20. Diagnostic criteria for chronic neutrophhilic leukaemia. (Continued) • Hepatosplenomegaly. • No identifiable cause for physiologic neutrophilia. No infectious or inflammatory process. No underlying tumour, or if present, demonstration of clonality of myeloid cells by cytogenetic or molecular studies. 5. No Philadelphia chromosome or BCR/ABL fusion gene.

  21. Diagnostic criteria for chronic neutrophhilic leukaemia. (Continued) • No evidence of another myeloproliferative disease. - No evidence of Polycythaemia Vera, i.e., normal red cell mass. - No evidence of chronic idiopathic myelofibrosis, i.e., no abnormal megakaryocytic proliferation, no reticulin or collagen fibrosis, no marked red blood cell poikilocytosis. - No evidence of essential thrombocythaemia, i.e., platelets <600X109/L, no proliferation of mature, enlarged megakaryocytes. • No evidence of a myelodysplastic syndrome or a myelodysplastic/myeloproliferative disorder. - No granulocytic dysplasia. - No myelodysplastic changes in other myeloid lineages. - Monocytes <1X109/L.

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