TY - JOUR
T1 - Pure red cell aplasia
T2 - Lymphocyte inhibition of erythropoiesis
AU - Abkowitz, J. L.
AU - Kadin, M. E.
AU - Powell, Jerry S
AU - Adamson, J. W.
PY - 1986
Y1 - 1986
N2 - The pathogens of pure red cell aplasia (PRCA) was studied in a patient who had no evidence of malignancy. In marrow culture, no erythroid colonies (from late erythroid progenitors [CFU-E]) but normal numbers of well-haemoglobinized erythroid bursts (from early erythroid progenitors [BFU-E]) were found, indicating that BFU-E existed in the patient but that their subsequent in vivo differentiation was inhibited. Autologous coculture studies suggested that inhibition was mediated by the patient's ER+ lymphocytes. After remission was induced with cyclophosphamide, autologous ER+ cells no longer suppressed in vitro erythropoiesis. However, cryopreserved ER+ cells, obtained with anaemia, suppressed BFU-E growth from remission marrow. An expanded population of large granular lymphocytes (LGL) with ER+, F(c)γ+, T3+, T8+, HNK-1+, Ia-, M1- phenotype and no functional natural killer (NK) cell activity was noted during PRCA that reverted to normal with remission. For this patient, both in vivo and in vitro evidence demonstrates a cellular inhibition of erythropoiesis at the level of differentiation between BFU-E and CFU-E.
AB - The pathogens of pure red cell aplasia (PRCA) was studied in a patient who had no evidence of malignancy. In marrow culture, no erythroid colonies (from late erythroid progenitors [CFU-E]) but normal numbers of well-haemoglobinized erythroid bursts (from early erythroid progenitors [BFU-E]) were found, indicating that BFU-E existed in the patient but that their subsequent in vivo differentiation was inhibited. Autologous coculture studies suggested that inhibition was mediated by the patient's ER+ lymphocytes. After remission was induced with cyclophosphamide, autologous ER+ cells no longer suppressed in vitro erythropoiesis. However, cryopreserved ER+ cells, obtained with anaemia, suppressed BFU-E growth from remission marrow. An expanded population of large granular lymphocytes (LGL) with ER+, F(c)γ+, T3+, T8+, HNK-1+, Ia-, M1- phenotype and no functional natural killer (NK) cell activity was noted during PRCA that reverted to normal with remission. For this patient, both in vivo and in vitro evidence demonstrates a cellular inhibition of erythropoiesis at the level of differentiation between BFU-E and CFU-E.
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M3 - Article
C2 - 3085703
AN - SCOPUS:0022636619
VL - 63
SP - 59
EP - 67
JO - British Journal of Haematology
JF - British Journal of Haematology
SN - 0007-1048
IS - 1
ER -