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Myeloproliferative disorder
- 1.
- 2. Definition Clonal neoplastic celldisorder of pluripotent hematopoietic stem cell characterized by excessive proliferation of one or more of the myeloid cell lineage.
- 3. Types 1. CML 2. Polycythemia 3.Essential thrombocythemia 4. Primary Myelofibrosis 5. Systemic Mastocytosis 6. Chronic eosinophilic leukemia 7. Stem cell leukemia
- 4. Characterized 1. Presence ofmutation 2. Constitutively activated tyrosine kinase 3. Growth factor independence 4. It does not impair the differentiation 5. Only the production increases
- 5. Common features 1. Increasedproliferation drive 2. Extra medullary hematopoiesis 3. Marrow fibrosis 4. Peripheral blood cytopenias 5. Transformation into acute leukemia's
- 6.
- 7. Definition • It isa type of myeloproliferative disorder in which the body produce many red blood cells as a result of problem with the bone marrow or increased production of erythropoietin hormone.
- 10. Pathogenesis JAK2 V617F Mutation Constitutiveactivation Activation of STAT/AKT/MAPK pathway Transcription activation Growth factor independent cell survival & proliferation
- 11. Morphology • Hypercellular bonemarrow • Increased red cell count • Increased granulocytic & megakaryocytic precursor cells • Peripheral smear • Increased red cell count • Increased basophils • Abnormally large platelets
- 12. Later stage • Progressto spent phase • Characterized by extensive marrow fibrosis • Increased extra medullary hematopoiesis • Transformation into AML in 1% cases
- 13. Clinical features • Appearsinsidiously • Adults • Symptoms are related to increased red cell count • Plethoric • Cyanotic • Headache • Dizziness • Hypertension • GI symptoms • Intense pruritis • Peptic ulceration Histamine released from basophils • Hyperuricemia
- 15. Complication 1. Bleeding manifestations 2.Thrombotic episodes
- 16. Investigation 1. Hematocrit =>60% 2. HB = 18 to 28 gm/dl 3. Peripheral smear 4. Bone marrow examination
- 17. Peripheral smear 1. Increasedred cell count 2. Increased white cell & platelets 3. Giant forms of platelets
- 18. Treatment 1. Phlebotomy 2. Radioactivephosphorous 3. Low dose aspirin 4. Anti histamine 5. H2 blockers 6. Ruxolitinib
- 19.
- 20. Introduction 1. Adults 2. 1to 3 /100000/year
- 21. DEFINITION • It isa type of myeloproliferative disorder in which mutation of JAK2 pathway leads to increased production of platelets in the bone marrow.
- 22. Pathogenesis 1. Mutation inJAK2 in 50% cases 2. Mutation in MPL in 5 to 10% 0f cases 3. Mutation in calreticulin 4. Increases the JAK2 / STAT signaling pathway 5. Constitute activation leads to Thrombopoietin independent proliferation
- 23. Characterized 1. Increased plateletcount 2. Absence of polycythemia 3. Absence of marrow fibrosis
- 24. Morphology • Hypercellular bonemarrow • Increased megakaryocyte • Abnormally large forms • Mild extra medullary hematopoiesis • Mild organomegaly
- 27.
- 28. Investigations 1. Peripheral smear 2.Bone marrow
- 30.
- 32. DEFINITION • It isa type of myeloproliferative disorder in which development of obliterative marrow fibrosis that leads to replacement of normal marrow by fibrous tissue which gives the reduced bone marrow hematopoiesis
- 33. Pathogenesis • Mutation ofJAK2 in 50 to 60 % cases • Activation of MPL in 1 to 5 % cases • Excessive deposition of collagen in the bone marrow • Due to inappropriate release of fibrogenic factors from neoplastic megakaryocytes
- 34. Morphology • Marrow • Early– Hyper cellular • Later – Hypocellular • Erythroid & granulocytic precursors are appear normal • Megakaryocyte appears large, dysplastic • Cloud like – clusters of atypical megakaryocyte with unusual nuclear shapes • Osteosclerosis • Extra medullary hematopoiesis • Cytopenias
- 35. Clinical features • Lesscommon • Adults • Usually 60 years • Anemia • Splenomegaly- fullness in the left upper quadrant • Fatigue • Weight loss • Night sweats • Hyperuricemia
- 36. Complications 1. Intercurrent infections 2.Thrombotic episodes 3. Bleeding tendency 4. Transform into AML in 5 to 20%
- 37. Investigations • Peripheral smear •Leukoerythyroblastosis – Premature release of nucleated Rbc’s & early granulocytic progenitors. • Tear drop shaped red cells • Bone marrow • Marrow fibrosis
- 38. Treatment 1. Chemotherapeutic agents 2.Hematopoietic stem cell transplantation