PMID: 6980901Sep 1, 1982Paper

Lymphocyte dysfunction in congenital hypoplastic anemia

The Journal of Clinical Investigation
J FinlayR Hong

Abstract

Congenital hypoplastic anemia (Diamond-Blackfan syndrome) is thought to involve the erythropoietic cell line alone. In this study, the evaluation of lymphocyte function in five patients with this syndrome revealed a number of abnormalities. Peripheral blood T lymphocyte percentages as assessed by monoclonal antibodies were decreased in three patients. T-helper/T-suppressor cell (OKT4:OKT8) ratios were almost unity in four of the five patients. We usually find a ratio of 2:1 in normal populations. Studies of lymphocyte-mediated suppression of lymphoproliferation demonstrated an inability to generate concanavalin A-induced suppressor cells in the same four patients and impaired prostaglandin-mediated suppression in two patients. Co-culture studies revealed a T lymphocyte-mediated suppression of erythropoiesis in a single patient, who also showed suppression of the mixed lymphocyte reaction. The four remaining patients showed no excessive suppressor effects either upon erythropoiesis or lymphoproliferation. These studies demonstrate that in congenital hypoplastic anemia, the cellular defect is not restricted to the erythroid progenitor cells, but extends to the lymphocytes.

References

Jan 1, 1979·American Journal of Hematology·B Löwenberg, H M De Zeeuw
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Citations

Dec 15, 1983·The New England Journal of Medicine·B E GladerL K Diamond
Mar 1, 1995·British Journal of Haematology·C P McGuckinE C Gordon-Smith
Jan 1, 1985·Critical Reviews in Oncology/hematology·A M Gewirtz, R Hoffman
Nov 11, 2010·Clinical and Experimental Immunology·S KhanA Huissoon
Jun 15, 2011·Pediatric Blood & Cancer·Andrzej RochowskiBlanche P Alter
Sep 1, 1989·British Journal of Haematology·P H TsaiJ M Lipton
Sep 1, 1988·British Journal of Haematology·M D'AvanzoF Iafusco
Oct 13, 2000·Baillière's Best Practice & Research. Clinical Haematology·M H Freedman
Apr 23, 2017·American Journal of Medical Genetics. Part a·Ebba AlkhunaiziNancy Braverman
Sep 1, 1985·Clinical Immunology and Immunopathology·A P Knutsen, D M O'Connor
Mar 1, 1992·American Journal of Medical Genetics·B A SemmekrotH G Brunner
Mar 1, 1994·Pediatric Hematology and Oncology·M D'AvanzoF Iafusco
Mar 29, 2014·Expert Review of Hematology·Adrianna VlachosJeffrey M Lipton
Aug 1, 1985·Scandinavian Journal of Haematology·K SawadaT Konno

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