In which of the following age group myelodysplastic syndrome (MDS) are most common: (AIIMS May 2009)
|C||20 – 40|
Clinical features of the myelodysplasia all relate to bone marrow failure.
1. MDS particularly affects the elderly (chronic myelomonocytic leukemia can occur in children)
2. Anemia features (progressive fatigue, dyspnea on exertion, pallor);
3. Neutropenia features (frequent infections);
4. Thrombocytopenia features (bleeding and bruising).
5. Splenomegaly in 20% cases
1. Laboratory findings include cytopenia, with anemia being the most frequent presenting feature.
2. Peripheral smear demonstrates (teardrop forms, Pelger Huët, hypogranulated, hypo lobular WBC forms, and abnormal platelet forms).
3. Bone marrow shows morphologic abnormalities including megaloblastic RBC, asynchronous maturation of cytoplasm and nuclei, ringed sideroblast forms, micromegakaryocytes, and excess blast forms.
4. In MDS – BM is hypercellular & normo cellular mainly but in 20% of cases it may be hypocellular.
Extra Edge: Pelger-Huët anomaly = It is characterized by a white blood cell type known as a neutrophil whose nucleus is hyposegmented.
MCQ: Essentials of Diagnosis
1. Cytopenias with a hypercellular bone marrow.
2. Morphologic abnormalities in two or more hematopoietic cell lines.
1. Supportive therapy: Erythropoietin (EPO) and G-CSF.
2. New agents a. Lenalidomide an immunomodularly agent, b. Azacytidine, an antimetabolite that impairs DNA methylation.