EXCESSIVE NAKED MEGAKARYOCYTE NUCLEI IN MYELODYSPLASTIC SYNDROME MIMICKING IDIOPATHIC THROMBOCYTOPENIC PURPURA: A Complicated Pre- and Post-transplantation Course

Abstract

A boy 3 years 7 months old with thrombocytopenia and history of intracranial hemorrhage who underwent bone marrow transplantation is presented. He was refractory to steroids, immunoglobulin G, vincristine, azathioprine, cyclosporine A, interleukin-11, chemotherapy, and splenectomy. Idiopathic thrombocytopenic purpura was excluded by light /electron microscopic and flow cytometric findings; the diagnosis of refractory cytopenia, a subgroup of pediatric myelodysplastic syndrome, was made. Naked megakaryocyte nuclei were 55.38 ± 28.2% vs. 31.67 ± 23.22% of all megakaryocytes in the patient and the control group of 9 patients with idiopathic thrombocytopenic purpura, respectively (p = .016). The posttransplatation course was complicated by delayed platelet engraftment, bronchiolitis obliterans associated with pneumocystis carinii pneumonia, which resolved completely.

Keywords :

• apoptosis
• dysmegakaryopoiesis
• myelodysplastic syndrome
• naked megakaryocyte nuclei
• refractory thrombocytopenia

ACKNOWLEDGMENT

This case was presented at the 4th International Symposium on MDS in Childhood, April 24–26, 2006, Freiburg, Germany.

Declaration of Interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.