1 / 21

Myeloid Mania

Goals and Objectives. Review myelodysplastic syndromesReview myeloproliferative disordersAnswer questionsAppreciate the intricacies and beauty of bone marrow and the process of hematopoiesis both as a fundamental means of maintaining homeostasis and an efficient use of otherwise undesirabl

merritt
Download Presentation

Myeloid Mania

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

    1. Myeloid Mania

    2. Goals and Objectives Review myelodysplastic syndromes Review myeloproliferative disorders Answer questions Appreciate the intricacies and beauty of bone marrow and the process of hematopoiesis both as a fundamental means of maintaining homeostasis and an efficient use of otherwise undesirable real estate

    3. Myelodysplastic Syndromes Pathogenesis Mutation in multi-potent stem cell Abnormal apoptosis Bottom line Ineffective hematopoiesis Complications Bleeding, infection (50%) End result Transformation to AML

    4. MDS: Diagnosis Average age: 65 History of exposure to Chemotherapy (alkylating agents) Radiation Hydrocarbons Xylene Toluene Agent Orange

    5. MDS: Diagnosis Bone marrow Peripheral smear Cytogenetics

    6. FAB Classification Refractory anemia Refractory anemia with ringed sideroblasts Refractory anemia with excess blasts Refractory anemia with excess blasts in transformation Now considered leukemia Chronic myelomonocytic leukemia Now classified as myelodysplastic- myeloproliferative overlap syndrome

    7. WHO Classification Refractory anemia Refractory anemia with ringed sideroblasts Refractory cytopenia with multilineage dysplasia Refractory cytopenia with multilineage dysplasia and ringed sideroblasts Refractory anemia with excess blasts I and II 5q- syndrome Myelodysplasia unclassifiable

    8. WHO Classification Refractory anemia (30-40%) < 5 % myeloblasts Median survival 27-50 months Refractory anemia with ringed sideroblasts (5-15%) < 5 % myeloblasts >15 % ringed sideroblasts Iron-laden mitochondria surrounding nucleus Median survival 40-50 months Treatment Chronic transfusion, EPO Bone marrow transplant Prognosis Least likely to progress

    9. WHO Classification Refractory cytopenia with multilineage dysplasia Bicytopenia or pancytopenia < 5 % blasts Dysplasia in > 10% of cells in 2-3 lineages < 1 x 10^9/L monocytes Refractory cytopenia with multilineage dysplasia and ringed sideroblasts Bicytopenia or pancytopenia < 5 % blasts Dysplasia in > 10% of cells in 2-3 lineages < 1 x 10^9/L monocytes > 15 % sideroblasts

    10. WHO Classification Refractory anemia with excess blasts (20-30%) Cytopenias Dysplasia of one or more lineages 5-9% blasts (I) 10-19% blasts (II) 5q- syndrome Women Myelodysplasia unclassifiable Megakaryocytic dysplasia with fibrosis

    11. MDS: Chemotherapy Azacitidine Pyrimidine nucleoside analogue Delays transformation Improves quality of life Lenalidomide Analogue of thalidomide 5q-

    12. Myeloproliferative Disorders Chronic myeloid leukemia Polycythemia vera Essential thrombocytopenia Myelofibrosis with myeloid metaplasia

    13. CML Pathogenesis t(9:22) Chronic (proliferative) phase Fatigue Weight loss Splenomegaly Thrombocytosis Accelerated phase Blast crisis ALL or AML

    14. CML: Treatment Imatinib Inhibits BCR-ABL tyrosine kinase 95% complete remission

    15. P. Vera Diagnosis A1. Hgb >18.5 (males, 16.5 (females) or 25% elevation A2. No secondary erythrocytosis A3. Splenomegaly A4. Clonal genetic abnormality A5. Endogenous erythroid colony formation in vitro B. Thrombocytosis (>400), bone marrow Bx with panmyelosis, leukocytosis (>12), low EPO Mutation: JAK2 (65-95%) Presentation Headache, pruritis, dyspnea, blurred vision, night sweats, facial plethora, splenomegaly

    16. P. Vera Prognosis Median survival 15 years Treatment Serial phlebotomy Hydroxyurea Aspirin

    17. ET Most common MPD Presentation Erythromelalgia Livedo reticularis Acral dysesthesia Headache Visual symptoms Arterial/venous thromboses Splenomegaly (50%)

    18. ET Diagnosis Platelets > 600,000 JAK2 (50%) Treatment None < 60 Asymptomatic No h/o thrombosis Platelets < 1,500,000 Hydroxyurea Goal: platelets < 400,000 Aspirin, plateletpheresis

    19. Myelofibrosis with Myeloid Metaplasia Pathogenesis Fibrosis-promoting cytokines Diagnosis Hepatosplenomegaly Extramedullary hematopoiesis Normocytic anemia Circulating erythroid and myeloid precursors Teardrop cells Bone marrow fibrosis

    20. Myelofibrosis with Myeloid Metaplasia Prognosis Median survival 3-5 years Complications Marrow failure Transformation to acute leukemia Portal hypertension Treatment Splenectomy Thalidomide +/- prednisone Bone marrow transplant

    21. MPD Recap Mutation / Gene / Treatment of CML? Philadelphia / BCR-ABL / imatinib CBC finding in P.vera? Hgb > 18.5 (males), 16.5 (females) Mutation / Treatment in ET? JAK2 (50%) / none Bone marrow finding in myelofibrosis Fibrosis

    22. the end

More Related