Delayed spontaneous remission in a child with primary acquired chronic pure red cell aplasia.

摘要: The acquired form of pure red cell aplasia (PRCA) presents either as an acute self-limited disease, predominantly seen in children, or a chronic illness more frequently adults with rare spontaneous remissions. A 14-year-old boy presented pallor, without hepatosplenomegaly, jaundice, lymphadenopathy, petechiae, any other apparent abnormalities. Isolated anemia the presence normal white and platelet counts marrow cellularity absence erythroblasts but myeloid cells megakaryocytes revealed diagnosis PRCA. All possible investigations excluded secondary causes patient required packed transfusions every 2 to 3 weeks. He failed therapy intravenous immunoglobulin, corticosteroids, cyclosporine plus antithymocyte globulin, anti-CD 20 (rituximab), erythropoietin (EPO). showed severe, resistant, transfusion-dependent Spontaneous remission hemoglobin reticulocyte levels was dramatic 6.5 years after PRCA 3.6 his last treatment.