Successful treatment of aplastic anemia–paroxysmal nocturnal hemoglobinuria associated with eosinophilic fasciitis with matched unrelated donor allogeneic peripheral blood stem cell transplantation
نویسندگان
چکیده
We report the first patient case of successful treatment intervention for both eosinophilic fasciitis and aplastic anemia with allogeneic peripheral blood stem cell transplantation from a matched unrelated donor after multiple immunosuppressant failure.
منابع مشابه
Allogeneic stem cell transplantation in paroxysmal nocturnal hemoglobinuria.
BACKGROUND In the era of eculizumab, identifying patients with paroxysmal nocturnal hemoglobinuria who may benefit from allogeneic stem cell transplantation is challenging. DESIGN AND METHODS We describe the characteristics and overall survival of 211 patients transplanted for paroxysmal nocturnal hemoglobinuria in 83 EBMT centers from 1978 to 2007. Next, we conducted a comparison with a coho...
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Paroxysmal nocturnal hemoglobinuria is caused by expansion of a hematopoietic stem cell clone with an acquired somatic mutation in the PIG-A gene. This mutation aborts the synthesis and expression of the glycosylphosphatidylinositol anchor proteins CD55 and CD59 on the surface of blood cells, thereby making them more susceptible to complement-mediated damage. A spectrum of disorders occurs in P...
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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...
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Transplantation of hematopoietic stem cells derived from bone marrow or peripheral blood has been used as a therapeutic procedure since the mid-seventies. In recent years, the number of transplants reported annually to the European Group for Blood and Marrow Transplantation (EBMT) Registry is approximately 23,500 including 38% of allogeneic and 62% of autologous procedures. In most developed co...
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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...
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