AMEGAKARYOCYTIC THROMBOCYTOPENIC PURPURA : A FIFTEEN YEAR EXPERIENCE

Amegakaryocytic thrombocytopenia, a rare variation of bone marrow aplasia, has been described in association with viral infection, vaccination, alcoholism and drug-toxicity. In the present study, we presented 20 cases of amegakaryocytic thrombocytopenic purpura in a cohort of Iranian patients presented to hematology clinics of two university hospitals with mucocutaneous bleeding. Complete blood counts, reticulocyte counts and repeated bone marrow aspiration/biopsy were carried out. Repeated bone marrow examinations revealed markedly decreased megakaryocytes but minimal or no decrease in myeloid and erythroid cell lines. Progression to aplastic anemia in 3 and progression to myelodysplasia/acute non lymphoblastic leukemia were observed in 2 patients. Two patients died from intracranial hemorrhage. In two patients splenectomy resulted in significant improvement in platelet counts and transfusion needs. Other patients had a fluctuating variable course with supportive care. It is suggested that splenectomy can be effective in reducing transfusion requirement in selected alloimmunized patients with acquired amegakaryocytic thrombocytopenia.