Posttransplantation thrombotic thrombocytopenic purpura: a single-center experience and a contemporary review.
نویسندگان
چکیده
OBJECTIVE To assess the activity of von Willebrand factor-cleaving protease (vWF-CP) in patients with thrombotic thrombocytopenic purpura (TTP) complicating bone marrow transplantation (BMT) and peripheral blood stem cell transplantation (PBSCT). PATIENTS AND METHODS From March 1, 1999, to June 30, 2001, allogeneic and autologous hematopoietic stem cell transplantation was performed in 118 and 400 patients, respectively. We reviewed risk factors for development of posttransplantation TTP and measured vWF-CP activity during active TTP in 10 recipients. RESULTS The incidence of TTP after allogeneic and autologous transplantation was 6.8% (8/118) and 0.25% (1/400), respectively. Among the allogeneic transplant recipients, the incidence of TTP after nonmyeloablative (NMA) PBSCT, matched unrelated donor BMT, and sibling BMT or PBSCT was 15.4% (2/13), 11.8% (2/17), and 4.5% (4/88), respectively. Of the 10 patients with TTP, 9 (90%) had received extensive prior therapy, including autologous transplantation in both NMA recipients. Acute graft-vs-host disease (GVHD) prophylaxis consisted of cyclosporine and methotrexate in most affected patients. The vWF antigen level was elevated in all patients, and no patients showed evidence of vWF-CP deficiency. During active TTP, 6 patients had grade II-IV acute GVHD, 1 had extensive chronic GVHD, and 4 had cytomegalovirus viremia. Risk factor analysis for development of TTP showed that transplant type (NMA and matched unrelated donor) and source of stem cells (bone marrow vs peripheral blood stem cell) were significant. CONCLUSIONS Posttransplantation TTP was not found to be associated with severe vWF-CP deficiency. The elevated levels of vWF antigen are consistent with diffuse endothelial injury likely because of multiple interacting factors such as extensive prior therapy, GVHD, cyclosporine, and reactivation of cytomegalovirus. The disorder appears to be more frequent among patients with, or at risk for, acute GVHD, suggesting a possible role in the pathogenesis. Nonmyeloablative transplantation does not appear to confer a lesser risk, possibly for these reasons.
منابع مشابه
Thrombotic Thrombocytopenic Purpura: Diagnosis and Treatment
Thrombotic Thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathic disease, associated with thrombocytopenia and hemolytic anemia. It is caused by an enzymatic dysfunction responsible in cleavage of blood clotting factors. In this study we have tried to review the available approaches in diagnoses of the disease as well as treatment strategies. Based on the what the current review ...
متن کاملSuccessful Treatment of Refractory Thrombotic Thrombocytopenic Purpura with Rituximab, a Monoclonal Antibody: a Case Report
Thrombotic thrombocytopenic purpura (TTP) is mostly attributed to the presence of an autoantibody against ADAMTS-13, a metalloprotease that degrades ultralarge von Willebrand protein multimers. Accumulation of vWF multimers and systemic platelet aggregation lead to microangiopathic thrombosis, hemolytic anemia, and end-organ ischemia. Most patients respond to therapeutic plasma exchange (TPE), ...
متن کاملReport of two unrelated cases of Familial Thrombotic Thrombocytopeic Purpura
Thrombotic Thrombocytopenic Purpura (TTP) is a rare microangiopathic disorder characterised by the pentad of microangiopathic hemolytic anemia, thrombocytopenic purpura, neurologic abnormalities, fever, and renal disease. Decreased production and/or activity of ADAMTS13 is the cause of this disorder. ADAMTS13 is a metalloproteinase which is responsible of the cleavage of high weight multimers o...
متن کاملA case of Acquired Thrombotic Thrombocytopenic Purpura without Renal Dysfunction
Objective: Thrombotic thrombocytopenic purpura (TTP) is the most malignant variant of microangiopathy that usually presents by typical symptoms including thrombocytopenia, hemolytic anemia, neurological abnormalities, fever and renal impairment. Report of the Case: We report a 12-year-old male presented by cytopenia, fever, purpura on his extremities, seizure and lethargy. Peripheral blood sm...
متن کاملA Case Report of Successful Management of Thrombotic Thrombocytopenic Purpura Syndrome (TTP) in a Pregnant Woman with Plasmapheresis
Background and Aim: Thrombotic thrombocytopenic purpura is a rare and life-threatening disease with a relatively high prevalence of 5% in pregnancy that causes thrombocytopenia. Due to the high prevalence and mortality of fetal and maternal mortality, which resolves rapidly with the onset of plasmapheresis, the diagnosis of this disease is clinically important. The aim of this study was to succ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Mayo Clinic proceedings
دوره 78 4 شماره
صفحات -
تاریخ انتشار 2003