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MLAB Hematology Keri Brophy-Martinez

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1 MLAB 1415- Hematology Keri Brophy-Martinez
Chapter 23: Myelodysplastic Syndromes

2 MYELODYSPLASTIC SYNDROMES
A group of acquired clonal hematologic disorders characterized by progressive cytopenias in the peripheral blood, reflecting defects in erythroid, myeloid and/or megakaryocytic maturation. Bone marrow is hyperplastic The result in impaired hematopoiesis. The origin is currently unknown. Affects people over 50 with a slight male predominance

3 Terms used to describe MDS
Preleukemic leukemia or syndrome Preleukemic acute human leukemia Chronic refractory anemia with sideroblasts Smoldering leukemia

4 Risk factors Age Genetic predisposition Chromosomes 5,7 and 8
Down’s, Fanconi’s Environmental exposures benzene Prior therapy Radiation Chemotherapy

5 Clinical findings Symptoms are related to progressive bone marrow failure Infections Bleeding Weight loss Cardiovascular Death usually occurs from infection and/or bleeding or from leukemia if transformation occurs.

6 Hematologic abnormalities in MDS
Findings Erythroid Myeloid Thrombocyte Peripheral blood Anemia Macro, oval macro,basophilic stippling, nRBC, Howell-Jolly bodies,sideroblasts aniso, poik Neutropenia Dysgranulopoiesis: Agranular or hypogranular neutrophils, Abnormal granulation of neutrophil precursors, Hyposegmented neutrophils Thrombocytopenia/thrombocytosis Hypogranulation Micromegakaryocytes Functional abnormalities Giant platelets Bone Marrow Megaloblastic erythroblasts nuclear fragments multiple nuclei vacuoles Abnormal granules in promyelocytes Absence of secondary granules Auer rods in blasts Megakaryocytes with multiple nuclei

7 Dysgranulopoiesis

8 FAB classification Five MDS subtypes: Refractory anemia (RA)
Refractory anemia with ringed sideroblasts (RARS) Refractory anemia with excess blasts (RAEB) Refractory anemia with excess blasts in transformation (RAEB-t) Chronic myelomonocytic leukemia (CMML)

9 WHO classification Refractory anemia (RA)
Anemia is primary finding, but unresponsive to treatment Refractory anemia with ringed sideroblasts (RARS) >15% ringed sideroblasts in bone marrow Refractory cytopenia with multilineage dysplasia (RCMD) Dysplastic features in at least 10% of cells in two or more lines RA with excess blasts-1 (RAEB) Cytopenia in at least two lineages, with qualitative abnormalities in all three cell lines

10 MDS/MPD Diseases Diseases
Clonal hematopoietic neoplasms that at initial presentation have some clinical, laboratory or morphological findings of both a MDS/MPD Abnormalities in the regulation of myeloid proliferation, maturation and cell survival Diseases CMML associated with persistent monocytosis Others

11 Chronic Myelomonocytic Leukemia (CMML)
Monocytosis ↑ in mature monocytes (some forms have ↑ monoblasts and promonocytes) Bone marrow hypercellular Splenomegaly and hepatomegaly (in patients with high WBC count)

12 Therapy Currently, there is no really good treatment for MDS, therefore most therapy is supportive, such as transfusion of blood components and antibiotics. Treatments that modulate the immune system, such as steroids and interferon have been used to some success. The only effective treatment is bone marrow transplant, but most patients are too old to survive the rigors of this treatment.

13 References McKenzie, Shirlyn B., and J. Lynne. Williams. "Chapter 21." Introduction. Clinical Laboratory Hematology. Boston: Pearson, Print


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