Clonality of acquired primary pure red cell aplasia.
Acquired primary pure red cell aplasia (PRCA) has frequently been shown to be associated with T cells that inhibit marrow erythropoiesis. A 41-year-old female presented with anemia in December 1985. Bone marrow examination revealed 1.8% erythroid cells. A diagnosis of PRCA was made. She received prednisolone, and her hemoglobin level recovered to 12 g/dl. In February 1995, her hemoglobin level decreased to 5.8 g/dl, and acquired primary PRCA recurred. Surface markers of peripheral blood mononuclear cells demonstrated CD4/8 ratio inversion. The T-cell receptor (TCR)-beta chain gene showed germ line configuration by Southern blot analysis of the mononuclear cells in peripheral blood. However, stepdown polymerase chain reaction analysis revealed that the TCR-beta gene of peripheral blood mononuclear cells was rearranged. With highly sensitive polymerase chain reaction analysis, clonality of T cells was confirmed. We propose that some acquired primary PRCA patients have a T-cell clonal disorder, similar to some PRCA patients with large granular lymphocytes leukemia or thymoma.
Masuda
M
M
Department of Hematology, Tokyo Women's Medical University, Tokyo, Japan.
Saitoh
H
H
Mizoguchi
H
H
D000328:Adult; D005260:Female; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; D019674:Genes, T-Cell Receptor beta; D006801:Humans; D016133:Polymerase Chain Reaction; D012010:Red-Cell Aplasia, Pure / Q000235:genetics / Q000276:immunology*