Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002878 (hemolytic anemia)
 7,530 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The case of a 27-year-old woman who simultaneously presented with SLE and severe refractory thrombotic microangiopathic hemolytic anemia (TMHA) is reported. She had extremely high levels of platelet-associated IgG (PAIgG), and her TMHA was refractory to plasma exchange and corticosteroid therapy. However, the TMHA was effectively controlled by i.v. cyclophosphamide therapy. ITP and TTP are generally considered distinct diseases; however, TMHA may occur secondary to platelet aggregation via autoimmune mechanisms in certain cases. Immunosuppressive therapy at an early stage of the disease may be beneficial in refractory cases of TMHA with autoimmune features.
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PMID:Intravenous cyclophosphamide therapy in a case with refractory thrombotic microangiopathic hemolytic anemia and SLE. 1580 Oct 75

Thrombotic thrombocytopenic purpura is a rare condition characterized by microangiopathic haemolytic anaemia, thrombocytopenia, altered neurology, renal impairment and fever. While plasma exchange has reduced mortality from more than 90% to between 10 and 30%, a proportion of cases fail to respond. Rituximab may be efficacious in the management of refractory cases of thrombotic thrombocytopenic purpura. We present two cases in which rituximab was used with successful outcomes. Treatment resulted in resolution of severe clinical and haematological abnormalities in both patients. There has been no relapse after 16 months follow up. Our experience supports the use of rituximab in difficult cases of TTP. Ongoing evaluation of its use is in progress at our institution.
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PMID:Anti-CD20 antibody in thrombotic thrombocytopenic purpura refractory to plasma exchange. 1750 82

Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP/HUS) is a multisystem disorder characterized by consumptive thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, renal function abnormalities, and fever. Coexistence of TTP/HUS and adult onset Still's disease (ASD) is extremely rare. We report the case of a 46-year-old woman who presented with fever, arthritis, myalgias, petechia on skin and confusion five years after the onset of ASD. Thrombocytopenia, renal failure, marked elevation lactate dehydrogenase, and red cell fragmentation on peripheral blood smear were observed. We made a diagnosis of TTP/HUS associated with ASD, according to physical examination and characteristic laboratory data. She recovered from the TTP/HUS following daily sessions of therapeutic plasma exchange with fresh frozen plasma replacement and glucocorticoid therapy. Awareness of the possible development of TTP/HUS in ASD is important for early diagnosis and treatment.
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PMID:Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome and adult onset Still's disease: case report and review of the literature. 1842 22

Thrombotic microangiopathy (TMA), a microvascular hemolytic disorder is a rare, but well described complication in organ transplant patients receiving immunosuppressant drugs. We report a 56-year-old female with a history of left lung transplant that presented to the hospital with microangiopathic hemolytic anemia and thrombocytopenia while receiving tacrolimus (FK 506) for 36 months. The patient was diagnosed with tacrolimus-induced TTP/HUS and started on daily plasmapheresis, and replacement of FK506 with cyclosporine. After thirteen plasmapheresis procedures, her platelet count, lactate dehydrogenase, and hematocrit were normalized. The ADAMTS-13 activity was subnormal and no inhibitory antibody was detected. The combination of daily plasmapheresis with fresh frozen plasma as a source of ADAMTS-13 and cyclosporine may be used as a rescue therapy in patients with FK506-induced TTP/HUS.
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PMID:Tacrolimus (FK506) associated thrombotic thrombocytopenic purpura/hemolytic uremic syndrome in lung transplant salvage with a plasmapheresis and cyclosporin. 1868 75

The Oklahoma thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) Registry, an inception cohort of 382 consecutive patients with TTP-HUS, provides a complete community perspective of these syndromes. TTP, as defined by thrombocytopenia and microangiopathic hemolytic anemia without an alternative etiology, is the appropriate term for all adults. These limited diagnostic criteria are supported by the presenting features of patients with ADAMTS13 deficiency, in whom both neurologic and renal abnormalities are uncommon. HUS is the appropriate term for children who fulfill these diagnostic criteria and who also have renal failure. These definitions are consistent with current management: plasma exchange is the essential treatment for most adults; supportive care is sufficient for children with HUS. Plasma exchange treatment has decreased the mortality of TTP from 90 to 10%. Patients with acquired autoimmune ADAMTS13 deficiency may also require immunosuppressive treatment to achieve a durable remission. Recovery has revealed previously unrecognized long-term risks. Recurrent acute episodes occur in approximately 40% of patients with acquired ADAMTS13 deficiency; most relapses occur within the first year and most patients have only one relapse. Adults with TTP of any etiology have a high risk for persistent minor cognitive abnormalities.
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PMID:The thrombotic thrombocytopenic purpura and hemolytic uremic syndromes: evaluation, management, and long-term outcomes experience of the Oklahoma TTP-HUS Registry, 1989-2007. 1918 Jan 37

Thrombotic thrombocytopenic purpura is an acute life threatening disorder, characterised by thrombocytopenia, microangiopathic haemolytic anaemia and multi organ microvascular thrombi that results in variable clinical symptoms. Just over a decade ago, the missing enzyme required for von Willebrand cleavage was recognised in TTP patients, subsequently identified as ADAMTS 13. Assays have confirmed that the majority of TTP cases are idiopathic and are associated with inhibitors and or IgG antibodies to ADAMTS 13. Such cases take longer to treat and are more likely to relapse. Evidence to date suggests the majority of antibodies block the spacer domain of ADAMTS 13. There may be other antibodies binding to ADAMTS 13 domains but their overall clinical involvement remains to be determined. Immunosuppressive treatments have until now been unsatisfactory. However, monoclonal anti-CD 20 therapy, acting on B-lymphocytes involved in antibody production results in remission in most patients and prevention of recurrent relapse. Further investigation into the antibodies produced in TTP and other aspects of immune dysfunction, such as T cells will further our understanding of this devastating disorder.
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PMID:Inhibitory anti-ADAMTS 13 antibodies: measurement and clinical application. 1996 8

Thrombotic microangiopathies are rare conditions characterized by microangiopathic hemolytic anemia, microthrombi, and multiorgan insult. The disorders, which include hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, are often acute and life threatening. We report a retrospective analysis of 65 patients presenting to our institution from 1997 to 2008 with all forms of thrombotic microangiopathy. Therapeutic plasma exchange was a requirement for analysis and 65 patients were referred to our institution; 66% of patients were female and median age at presentation was 52 years. Bacterial infection was the most commonly identified etiologic factor and in the multivariate model was the only significant variable associated with survival outcome (odds ratio 5.1, 95% confidence interval, 1.2-21.7). As infection can be considered a common trigger event for thrombotic microangiopathy, patients with hepatobiliary sepsis may benefit from elective cholecystectomy. We conclude that bacterial infection frequently triggers TTP and other thrombotic microangiopathies in patients with preexisting risk factors and propose a model for the development of these syndromes.
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PMID:Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors: a single-institution experience. 2010 77

Sunitinib, a new vascular endothelial growth factor receptor inhibitor, has demonstrated activity in renal cell carcinoma and is now widely used in the palliative treatment of patients with metastatic renal cell carcinoma. It is generally well tolerated but has been associated with a low incidence of grade 3 and 4 toxicities including fatigue, diarrhea, anorexia, mucositis, skin toxicity, immune thrombocytopenic purpura, hypertension, hypothyroidism, cytopenias, and decreased cardiac ejection fraction. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is a rare condition that is severe and may be fatal. Several medications have been implicated in causing TTP-HUS including clopidogrel, mitomycin C, cisplatin. In this report, we describe a case of atypical HUS-microangiopathic hemolytic anemia during treatment with sunitinib in a patient with metastatic renal cell carcinoma. To our knowledge, this is the fourth case of microangiopathic hemolytic anemia associated with sunitinib described in the literature and the first case with fatal outcome despite treatment with plasmapheresis, dialysis, and withdrawal of sunitinib.
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PMID:Sunitinib-induced microangiopathic hemolytic anemia with fatal outcome. 2140 68

Endothelial injury is perhaps the inciting factor leading to the microangiopathic process that initiates thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS). TTP-HUS after postendoscopic retrograde cholangiopancreatography (ERCP) pancreatitis is extremely rare, but potentially is life threatening. Here, we describe a case of a 23-year-old man with a history of choledocholithiasis, who developed TTP-HUS, 2 days after the onset of post-ERCP pancreatitis. It is important that physicians recognize TTP-HUS as one of the potential causes of acute kidney injury in cases of acute pancreatitis and post-ERCP pancreatitis for adult patients, especially when there is concomitant thrombocytopenia and hemolytic anemia. The early initiation of plasma exchange has a major impact on the survival and preservation of renal function. Exchange transfusion of fresh frozen plasma remains the cornerstone treatment of TTP-HUS.
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PMID:Postendoscopic retrograde cholangiopancreatography pancreatitis: a rare cause of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. 2171 16

Thrombotic microangiopathy results from thrombotic occlusion of the microvasculature leading to fragmentation of red blood cells, profound thrombocytopenia, and a microangiopathic hemolytic anemia with elevation of lactate dehydrogenase and negative direct Coomb's test. This constellation of clinical and laboratory findings is not due to one disease entity; rather, it represents a variety of underlying diagnoses. Among the major disease entities are TTP/HUS, which can be congenital or acquired, bacterial infections, medications, vascular or endothelial pathology like Kasabach-Merritt phenomenon, and stem cell transplantation. In this paper, we offer a review of some of the major causes of thrombotic microangiopathy.
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PMID:Thrombotic microangiopathies. 2288 46


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