Pure red cell aplasia: Lymphocyte inhibition of erythropoiesis

摘要: Summary The pathogenesis of pure red cell aplasia (PRCA) was studied in a patient who had no evidence malignancy. In marrow culture, erythroid colonies (from late progenitors [CFU-E]) but normal numbers well-haemoglobinized bursts early [BFU-E]) were found, indicating that BFU-E existed the their subsequent vivo differentiation inhibited. Autologous coculture studies suggested inhibition mediated by patient's ER+ lymphocytes. After remission induced with cyclophosphamide, autologous ER + cells longer suppressed vitro erythropoiesis. However, cryopreserved cells, obtained anaemia, growth from marrow. An expanded population large granular lymphocytes (LGL) +, Fcγ+. T3 T8 HNK-1 Ia —, M1 — phenotype and functional natural killer (NK) activity noted during PRCA reverted to remission. For this patient, both demonstrates cellular erythropoiesis at level between CFU-E.