Serum Anti-YG Globulin Factors in Paroxysmal Nocturnal Hemoglobinuria
نویسندگان
چکیده
I N PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH) accelerated destruction of abnormal red cells occurs within an apparently benign extracorpuscular environment.1 Although the underlying cellular defect has not yet been fully elucidated, it has long been recognized that, in vitro, PNII erythrocytes are extraordinarily susceptible to immune lysis. The possibility exists that, in vivo, destruction of the PNH red cell may be provoked by immunologic mechanisms. However, evidence for such a process is usually lacking,2 and to the present time there has been no consistently recognized abnormahity in the serum globulins or in serum complement activity. It was, therefore, of considerable interest that serum from a patiemit with PNH was found to agglutinate compatible red cells that had been sensitized with some incomplete Rh antisera. This finding suggested that the patient’s serum contained rheumatoid factor ( RF). However, neither the patient’s history nor physical findings suggested the diagnosis of rheumatoid arthritis. Since a search of the literature revealed no previously noted association between PNH and anti-yG globulin factors, additional sera from PNH patients were sought; a total of 25 such patients were finally collected for study.#{176} The results of this investigation, previously reported in preliminary form,4 indicate that anti-yG globulin factors are commonly present in the sera of patients with PNH.
منابع مشابه
Paroxysmal nocturnal hemoglobinuria clones in severe aplastic anemia patients treated with horse anti-thymocyte globulin plus cyclosporine.
BACKGROUND Clones of glycosylphosphatidylinositol-anchor protein-deficient cells are characteristic in paroxysmal nocturnal hemoglobinuria and are present in about 40-50% of patients with severe aplastic anemia. Flow cytometry has allowed for sensitive and precise measurement of glycosylphosphatidylinositol-anchor protein-deficient red blood cells and neutrophils in severe aplastic anemia. DE...
متن کاملBone marrow transplantation for paroxysmal nocturnal hemoglobinuria: eradication of the PNH clone and documentation of complete lymphohematopoietic engraftment.
Paroxysmal nocturnal hemoglobinuria (PNH) involves the proliferation of an abnormal and possibly premalignant hematopoietic stem cell. Successful treatment of PNH by marrow grafting requires that the PNH clone be eradicated by the pretransplant conditioning regimen. Four patients with PNH-associated marrow aplasia were transplanted with marrow from their HLA-matched, MLR-nonreactive siblings. T...
متن کاملIntestinal perforation in a patient with paroxysmal nocturnal hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias.Acute abdominal pain is one of the PNH clinical manifestations due to venous thrombosis of intra-abdominal sites including hepatic, portal, mesenteric, and splenic veins.Eculizumaband allogeneic bone marrow transplantation (BMT) arethe only w...
متن کاملThe Immune-mediated Pathogenesis and Treatment of Aplastic Anemia
Aplastic anemia is an immune-mediated disorder in most cases. The suppression of hematopoiesis by CD8+ T cells and also the overproduction of interferon(IFN)-γ and tumor necrosis factor (TNF)-α due to the Th1 response were demonstrated. Aplastic anemia patients with HLA-DRB1*1501 and oligoclonality of the T-cell repertoire in the peripheral blood showed immunosuppressive therapy (IST), cyclospo...
متن کاملLate complications following treatment for severe aplastic anemia (SAA) with high-dose cyclophosphamide (Cy): follow-up of a randomized trial.
High-dose cyclophosphamide (Cy) has been promoted as curative therapy for severe aplastic anemia (SAA). However, our randomized trial comparing antithymocyte globulin (ATG) and Cy was terminated early because of excess morbidity/early mortality in the Cy arm. We now report analysis of secondary endpoints at a median of 38 months. Relapse occurred in 6 (46%) of 13 responders in the ATG arm versu...
متن کامل