Microscopy of a specimen shows “pawn ball megakaryocytes”. This patient may have:
Correct Answer: Myelodysplastic syndrome
Description: Myelodysplastic syndromes (MDS) are a group of clonal stem cell disorders characterized by maturation defects associated with ineffective erythropoiesis and a high risk of transformation to AML. The most characteristic finding is disordered differentiating affecting all three lineages i.e., erythroid, myeloid, and megakaryocytic. Erythroid lineage effects: Ringed sideroblasts,that is erythroblasts with iron-laden mitochondria visible as perinuclear granules on Prussian blue stain Megaloblastoid maturation, resembling that seen in vitamin B12 or folate deficiency Nuclear budding abnormalities, producing misshapen nuclei Granulocyte lineage effects: Neutrophils with decreased numbers of secondary granules, toxic granulations, or Dohle bodies Pseudo-Pelger-Huet cells (neutrophils with only two nuclear lobes) Myeloblasts may be increased but account for less than 20% of overall marrow cellularity Megakaryocytic lineage effects: Megakaryocytes with single nuclear lobes or multiple separate nuclei ("pawn ball" megakaryocytes Ref: Pocket Companion to Robbins & Cotran Pathologic Basis of Disease By Richard Mitchell, Vinay Kumar, Nelson Fausto, Abul K. Abbas, Jon C. Aster, 8th edition, Page 334-335.
Category:
Pediatrics
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