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Myelodysplastic Syndrome. Group of clonal disorders of hematopoietic stem cells characterized by cellular dysplasia and ineffective hematopoiesis Group.

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Presentation on theme: "Myelodysplastic Syndrome. Group of clonal disorders of hematopoietic stem cells characterized by cellular dysplasia and ineffective hematopoiesis Group."— Presentation transcript:

1 Myelodysplastic Syndrome

2 Group of clonal disorders of hematopoietic stem cells characterized by cellular dysplasia and ineffective hematopoiesis Group of clonal disorders of hematopoietic stem cells characterized by cellular dysplasia and ineffective hematopoiesis Results in cytopenias and dysmyelopoiesis Results in cytopenias and dysmyelopoiesis Variable risk of transformation to acute leukemia Variable risk of transformation to acute leukemia

3 Pathophysiology Primary MDS or Secondary MDS Primary MDS or Secondary MDS A clonal mutation in hematopoietic stem cells A clonal mutation in hematopoietic stem cells Bone marrow failure due to ineffective hematopoiesis Bone marrow failure due to ineffective hematopoiesis Increased cytokines (TNF-a) inhibit hematopoiesis Increased cytokines (TNF-a) inhibit hematopoiesis Increased apoptosis Increased apoptosis Results in futile cycling of blood cells with impaired production of mature blood cells Results in futile cycling of blood cells with impaired production of mature blood cells

4 Prevalence Mainly a disorder of older patients, rarely presents before age 50 Mainly a disorder of older patients, rarely presents before age 50 Median age is 65 years Median age is 65 years Male predominance Male predominance

5 Clinical Presentation Non specific signs and symptoms of various cytopenias Fatigue, SOB, exercise intolerance, pallor, tachycardia Fatigue, SOB, exercise intolerance, pallor, tachycardia Mucosal bleeding, petechiae, ecchymoses Mucosal bleeding, petechiae, ecchymoses Infection, fever Infection, fever B-symptoms are uncommon Splenomegaly and lymphadenopathy are rare

6 Diagnosis Anemia uniformly present with decreased reticulocytosis Anemia uniformly present with decreased reticulocytosis >50% present with pancytopenia >50% present with pancytopenia 50% present with associated neutropenia 50% present with associated neutropenia <5% isolated neutropenia or thrombocytopenia <5% isolated neutropenia or thrombocytopenia Cytopenia with normal or hypercellular bone marrow with single or multi-lineage dysplasia Cytopenia with normal or hypercellular bone marrow with single or multi-lineage dysplasia

7 Morphology Peripheral changes include oval macrocytic red cells, hypogranular granulocytes with the pseudo-Pelger–Huët anomaly, and giant platelets. Peripheral changes include oval macrocytic red cells, hypogranular granulocytes with the pseudo-Pelger–Huët anomaly, and giant platelets.

8 Morphology Megaloblastic red-cell precursors with multiple nuclei or asynchronous maturation of the nucleus and the cytoplasm Megaloblastic red-cell precursors with multiple nuclei or asynchronous maturation of the nucleus and the cytoplasm

9 Morphology Ringed sideroblasts, erythroid precursors with iron-laden mitochondria Ringed sideroblasts, erythroid precursors with iron-laden mitochondria

10 Classification Disease Peripheral Blood Bone Marrow Refractory Anemia (RA) Anemia No or rare blasts Erythroid dysplasia only < 5% blasts < 15% ringed sideroblasts Refractory Anemia with Ringed Sideroblasts (RARS) Anemia No blasts Erythroid dysplasia only >15% ringed sideroblasts < 5% blasts Refractory cytopenia with multilineage dysplasia (RCMD) Cytopenia No or rare blasts No Auer rods < 1 × 10 9 /L monocytes Dysplasia in 10% of cells in 2 or more myeloid cell lines < 5% blasts in marrow < 15% ringed sideroblasts

11 Classification Cont. DiseasePeripheral BloodBone Marrow Refractory cytopenia with multilineage dysplasia and ringed sideroblasts (RCMD-RS) Cytopenia No or rare blasts No Auer rods < 1 × 10 9 /L monocytes Dysplasia in 10% of cells in 2 or more myeloid cell lines 15% ringed sideroblasts Refractory anemia with excess blasts-1 (RAEB-1) Cytopenias < 5% blasts No Auer rods < 1 × 109/L monocytes Unilineage or multilineage dysplasia 5% to 9% blasts No Auer rods Refractory anemia with excess blasts-2 (RAEB-2) Cytopenias 5% to 19% blasts Auer rods ± < 1 × 109/L monocytes Unilineage or multilineage dysplasia 10% to 19% blasts ±Auer rods

12 Classification cont. Disease Peripheral Blood Bone Marrow Myelodysplastic syndrome, unclassified (MDS-U) Cytopenias No or rare blasts No Auer rods Unilineage dysplasia in granulocytes or megakaryocytes < 5% blasts No Auer rods MDS associated with isolated del(5q) Anemia < 5% blasts Platelets normal or increased Normal to increased megakaryocytes with hypolobated nuclei < 5% blasts No Auer rods Isolated del(5q)


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