[Glycosilphosphatidylinositol-anchored cell surface protein deficiency in Mexican mestizo patients with aplastic anemia].
The peripheral blood cells of ten patients with biopsy-proven aplastic anemia were studied by means of flow-cytometry in order to assess the expression of two phosphatidylinositol-anchored surface proteins: CD55/DAF (decay accelerating factor) and CD59/MIRL (membrane inhibitor of reactive lysis). An abnormal expression was found in five of these ten patients, whereas the "traditional" tests for paroxysmal nocturnal hemoglobinuria (PNH) were positive only on two of these five individuals. Five of the aplastic patients were treated with anti-thymocyte globulin and cyclosporin-A and three entered a complete remission; of the latter, one had CD55/CD59 deficiencies whereas two did not. Along the study period one patient with a hemolytic pattern of PNH was identified. It is concluded that CD55 and/or CD59 abnormalities are frequent in Mexican mestizo patients with aplastic anemia, that the aplastic presentation of PNH is more frequent in Mexico than the hemolytic presentation, that the flow-cytometric identification of CPI-anchored proteins is more sensitive than the "traditional" PNH tests, and that some patients with PNH-aplasia may respond to intensive immunosuppressive treatment. The flow-cytometric identification of GPI-anchored cell surface proteins should replace the "traditional" tests in the identification of patients with PNH.
Ruiz-Argüelles
G J
GJ
Centro de Hematología y Medicina Interna de Puebla, México. gruiz1@pue1.telmex.net.mx
Ramírez Cisneros
F J
FJ
Ruiz-Argüelles
A
A
Pérez-Romano
B
B
Morales-Aceves
R
R
D000741:Anemia, Aplastic / Q000097:blood* / Q000150:complications / Q000208:ethnology; D018958:CD55 Antigens / Q000097:blood*; D018957:CD59 Antigens / Q000097:blood*; D005434:Flow Cytometry; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood / Q000150:complications / Q000175:diagnosis; D006801:Humans; D007198:Indians, North American; D008800:Mexico
D018958:CD55 Antigens; D018957:CD59 Antigens