Myeloprolifirative disorders 3.57.51 PM

Myeloprolifirative disorders 3.57.51 PM -...

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Myeloproliferative disorders Dr. Tariq Roshan PPSP Department of Hematology
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Introduction Hemopoietic stem cell disorder Clonal Characterized by proliferation Granulocytic Erythroid Megakaryocytic Interrelationship between Polycythaemia Essential thrombocythaemia myelofibrosis
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Introduction / haemopoiesis
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Introduction Normal maturation (effective) Increased number of Red cells Granulocytes Platelets (Note: myeloproliferation in myelodysplastic syndrome is ineffective) Frequent overlap of the clinical, laboratory & morphologic findings Leucocytosis, thrombocytosis, increased megakaeryocytes, fibrosis & organomegaly blurs the boundaries Hepatosplenomegaly Sequestration of excess blood Extramedullary haematopoiesis Leukaemic infiltration
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Rationale for classification Classification is based on the lineage of the predominant proliferation Level of marrow fibrosis Clinical and laboratory data (FBP, BM, cytogenetic & molecular genetic)
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Differential diagnosis Disease BM cellularity % marrow blasts Maturation Morphology Haemato -poiesis Blood counts Large organs MPD Increased Normal or < 10% Present Normal Effective One or more myeloid increased Common MDS Usually increased Normal or < 20% Present Abnormal In- effective Low one or more cytopenia Un- common MDS/ MPD Usually increased Normal or <20% Present Abnormal Effective or in- effective Variable Common AML Usually increased Increased >20% Minimal Dysplasia can be present In- effective Variable Un- common
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Clonal evolution Clonal evolution & stepwise progression to fibrosis, marrow failure or acute blast phase
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Incidence and epidemiology Disease of adult Peak incidence in 7 th decade 6-9/100,000
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This note was uploaded on 12/24/2011 for the course STEP 1 taught by Professor Dr.aslam during the Fall '11 term at Montgomery College.

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Myeloprolifirative disorders 3.57.51 PM -...

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