Chapter 14 The Myelodysplastic Syndromes Myelodysplastic Syndromes MDSs Abnormal clone of cells Stem cell disorder results in a neoplasm May be caused by chromosomal abnormality Two types of MDSs De novo Unrelated to other treatment Secondary Related to prior treatment Myelodysplastic Syndromes MDSs Symptoms Hypercellular marrow Macrocytic anemia Weakness Infection Easy bruisability Diagnosis based on Percentage of blasts Type of dysplasia Ringed sideroblasts Myelodysplastic Syndromes MDSs Classification into one of the six subtypes of MDS based on Marrow Peripheral smear Cytogenetic studies goes back to karyotype Immunologic features How to Recognize Dysplasia Dysplasia is an abnormal development of tissue Clinical findings include Multinuclearity Disintegration of the nucleus Asynchrony similar to megaloblastic changes Nuclear bridging between cells Vacuolization or poor granulation Hypogranulated hyposegmented or hypergranulated cells Pseudo Pelger Huet cells Red cell size changes Platelet abnormalities in the peripheral smear include abnormal size Abnormal granulation Megakaryocytic fragments Classification of the Myelodysplastic Syndrome Classification devised by FrenchAmerican British FAB investigative group World Health Organization WHO revised the work of FAB WHO classification based on additional knowledge gained from molecular immunologic and cytogenetic studies Specific Features of the WHO Classification Refractory anemia RA Erythroid hyperplasia Refractory anemia with ringed sideroblasts RARS Refractory anemia in which 15 or more of red cell precursors are ringed sideroblasts Refractory anemia with multilineage dysplasia Bone marrow failure with two or more myeloid cell lines affected Specific Features of the WHO Classification Refractory anemia with excess blasts RAEB Shows anemia thrombocytopenia and neutropenia Hypercellular marrow in most cases Myelodysplastic syndrome unclassifiable Features of MDS but not a clear classification Neutropenia and thrombocytopenia are common Deleted 5q Deletion of the long arm of chromosome 5 Normal or elevated platelet counts are seen
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