IngentaConnect Deficiency of glycosylphosphatidyl inositol-anchored proteins in ...

Authors: Lord, Corinne De¹; Tooze, Jennifer A.¹; Saso, Radovan¹; Marsh, Judith C. W.¹; Gordon-Smith, Edward C.¹

Source: British Journal of Haematology, Volume 101, Number 1, April 1998 , pp. 90-93(4)

Abstract:

Deficient expression of glycosylphosphatidyl inositol (GPI)-anchored proteins in aplastic anaemia (AA) patients has previously been reported to be associated with a poor response to immunosuppressive (IS) therapy. Here we report the response to IS therapy of 111 patients with AA and correlate this with GPI-anchored protein expression on peripheral blood cells by flow cytometry. A GPI-anchored protein deficient population was identified in 15% (17/111) of patients with AA who had a negative Ham's test and no laboratory evidence of haemolysis. Patients were treated with antilymphocyte globulin and/or cyclosporin A, or oxymetholone. Bone marrow transplantation was performed in 12 patients, seven of whom had not responded to IS therapy. In patients tested for GPI-anchored protein expression prior to IS therapy there was no difference in response rate to IS therapy between AA patients with a GPI-anchored protein deficiency and those with normal GPI-anchored protein expression (50% response rate versus 75%, respectively). Survival in these two groups was similar at 90% with follow-up over 140 months from diagnosis. Eight of the 17 AA patients who developed a GPI-anchored protein-deficient population later went on to develop a positive Ham's test. From this study we demonstrate a lower incidence of GPI-anchored protein deficiency in AA patients compared with previous reports. In addition we have shown that the presence of a GPI-anchored protein-deficient cell population in patients with AA who have a negative Ham's test is not a poor prognostic factor in terms of response and survival after IS therapy.

Keywords: aplastic anaemia; GPI-anchored proteins; immunosuppressive therapy; response

Document Type: Original article

DOI: 10.1046/j.1365-2141.1998.00660.x

Affiliations: 1: Division of Haematology, Department of Cellular and Molecular Sciences, St George's Hospital Medical School, London

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Deficiency of glycosylphosphatidyl inositol-anchored proteins in patients with aplastic anaemia does not affect response to immunosuppressive therapy