PubMed Journals Articles About "Relapse Rate Survivors Acute Autoimmune Thrombotic Thrombocytopenic Purpura" RSS

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Showing "Relapse Rate Survivors Acute Autoimmune Thrombotic Thrombocytopenic Purpura" PubMed Articles 1–25 of 19,000+

Relapse Rate in Survivors of Acute Autoimmune Thrombotic Thrombocytopenic Purpura Treated with or without Rituximab.

 Autoimmune thrombotic thrombocytopenic purpura (iTTP) is caused by autoantibody-mediated severe a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13 (ADAMTS13) deficiency leading to micro-angiopathic haemolytic anaemia (MAHA) and thrombocytopenia with organ damage. Patients survive with plasma exchange (PEX), fresh frozen plasma replacement and corticosteroid treatment. Anti-CD20 monoclonal antibody rituximab is increasingly used in patients resistant to conventional PEX or rel...

Prevention of relapse in patients with acquired thrombotic thrombocytopenic purpura undergoing elective surgery: a case series.

Severe ADAMTS13 deficiency has been recognized as the main risk factor for recurrence of thrombotic thrombocytopenic purpura (TTP). Several conditions including surgery may influence the levels of ultra large Von Willebrand factor and ADAMTS13, acting as a trigger for an acute TTP event.

Relapsed/refractory acquired thrombotic thrombocytopenic purpura in a patient with Sjögren syndrome: Case report and review of the literature.

Thrombotic thrombocytopenic purpura (TTP) is a rare, fatal disorder which could be caused by autoimmune diseases. However, TTP secondary to Sjögren syndrome (SS) is extremely rare.

Thrombotic Microangiopathy, Hemolytic Uremic Syndrome, and Thrombotic Thrombocytopenic Purpura Following Hump-nosed Pit Viper (Genus: Hypnale) Envenoming in Sri Lanka.

Thrombotic microangiopathy (TMA), which includes the spectrum of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, is an uncommon complication of hump-nosed pit viper envenomation. We describe 4 cases of TMA following hump-nosed pit viper (Hypnale spp) bites in Sri Lanka. The first case is a typical TMA that spontaneously resolved with supportive treatments. The second and third cases are related to hemolytic uremic syndrome complicated with acute kidney injury that required hemodialysis. T...

Acquired autoimmune thrombotic thrombocytopenic purpura.

Clinical presentation in thrombotic thrombocytopenic purpura: Real-world data from two Mexican institutions.

Thrombotic thrombocytopenic purpura (TTP) is a hematologic disease that can be fatal if not treated early. We aimed to describe the clinical characteristics of Mexican patients with idiopathic TTP.

Thrombotic thrombocytopenic purpura and hemophagocytic lymphohistiocytosis in an elderly man: A case report.

Thrombotic thrombocytopenic purpura (TTP) and hemophagocytic lymphohistiocytosis (HLH) are rare hematologic conditions and have high mortality. Both TTP and HLH result from deregulation of the immune system. There are no published reports of coexisting TTP and HLH in elderly patients.

Validation of the PLASMIC score, a clinical prediction tool for thrombotic thrombocytopenic purpura diagnosis, in Chinese patients.

The recently published PLASMIC score was shown to be an excellent diagnostic model to identify patients with severe ADAMTS-13 deficiency. However, it is unclear if this score is suitable for Chinese patients with suspected thrombotic thrombocytopenic purpura (TTP).

Link between von Willebrand factor multimers, relapses and coronary microcirculation in patients with thrombotic thrombocytopenic purpura in remission.

ADAMTS13 deficiency results in unusually large von Willebrand factor (ULVWF) multimers in the circulation and a higher risk of microthrombi due to high shear stress. In patients treated for acquired thrombotic thrombocytopenic purpura (TTP), a persistently severe ADAMTS13 deficiency (

Clinical and Laboratory Features of Patients with Acquired Thrombotic Thrombocytopaenic Purpura: Fourteen Years of the Milan TTP Registry.

Acquired thrombotic thrombocytopaenic purpura (TTP) is a rare thrombotic microangiopathy caused by the immune-mediated severe deficiency of ADAMTS13. We hereby report the demographic and disease-related data of acquired TTP patients recorded in the Milan TTP Registry ( We performed a cross-sectional study of 302 individuals enrolled in our registry for an acute episode of acquired TTP occurred between 2002 and 2015 (female 77%; median age at onset 40 years, interquartile range: 30-50). ...

Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura.

In acquired thrombotic thrombocytopenic purpura (TTP), an immune-mediated deficiency of the von Willebrand factor-cleaving protease ADAMTS13 allows unrestrained adhesion of von Willebrand factor multimers to platelets and microthrombosis, which result in thrombocytopenia, hemolytic anemia, and tissue ischemia. Caplacizumab, an anti-von Willebrand factor humanized, bivalent variable-domain-only immunoglobulin fragment, inhibits interaction between von Willebrand factor multimers and platelets.

Thrombotic thrombocytopenic purpura complicated with acute erythropoiesis dysfunction: a case report and literature review.

Thrombotic thrombocytopenic purpura associated with dengue and chikungunya virus coinfection: case report during an epidemic period.

The present report shows the occurrence of thrombotic thrombocytopenic purpura (TTP) associated with acute dengue and chikungunya virus coinfection, manifesting as a severe disease with high mortality potential. The patient was a 28 year-old man with clinical and epidemiological diagnosis of arboviruses infections who developed thrombocytopenia and anemia, after which oral corticosteroid therapy was started. On the third day of hospitalization, he showed neurological alterations that simulated a cerebral va...

No more thrombotic thrombocytopenic purpura/hemolytic uremic syndrome please.

Secondary thrombotic microangiopathy with severely reduced ADAMTS13 activity in a patient with Capnocytophaga canimorsus sepsis: a case report.

The Gram-negative bacillus Capnocytophaga canimorsus may cause a severe illness resembling thrombotic thrombocytopenic purpura (TTP). The pathogenesis and optimal therapy of this secondary thrombotic microangiopathy (TMA) remain uncertain.

Vitamin B deficiency-induced pseudothrombotic microangiopathy without macrocytosis presenting with acute renal failure: a case report.

Vitamin B deficiency-induced thrombotic microangiopathy, known as pseudothrombotic microangiopathy, is a rare condition which resembles the clinical features of thrombotic thrombocytopenic purpura but requires a markedly different treatment. Most cases of vitamin B deficiency have only mild hematological findings, but in approximately 10% of patients life-threatening conditions have been reported.

Impact of a multidisciplinary team for the management of thrombotic microangiopathy.

Thrombotic microangiopathy (TMA) is an important complication associated with several diseases that are rare and life-threatening. TMA is common to thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). TTP is defined by a severe deficiency of ADAMTS13, and early treatment is associated with good prognosis. The diagnosis of HUS can be difficult due to the potential multiple etiologies, and the best treatment option in most cases is not well-established yet. The implementation of a mu...

HIV-associated thrombotic thrombocytopaenic purpura: A retrospective cohort study during the anti-retroviral therapy era.

HIV-associated thrombotic thrombocytopaenic purpura (TTP) is thought to represent the majority of current TTP diagnoses in South Africa (SA).

Correction to: Transfusion of Platelets Loaded With Recombinant ADAMTS13 (A Disintegrin and Metalloprotease With Thrombospondin Type 1 Repeats-13) Is Efficacious for Inhibiting Arterial Thrombosis Associated With Thrombotic Thrombocytopenic Purpura.

Anti-ADAMTS13 Autoantibodies against Cryptic Epitopes in Immune-Mediated Thrombotic Thrombocytopenic Purpura.

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is characterized by severe ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) deficiency, the presence of anti-ADAMTS13 autoantibodies and an open ADAMTS13 conformation with a cryptic epitope in the spacer domain exposed. A detailed knowledge of anti-ADAMTS13 autoantibodies will help identifying pathogenic antibodies and elucidating the cause of ADAMTS13 deficiency. We aimed at cloning anti-ADAMTS13 autoantibo...

Distinguishing autoimmune myelofibrosis from primary myelofibrosis.

Bone marrow fibrosis (BMF) is a histologic finding in a wide range of diseases, including malignancies, endocrine disorders, autoimmune diseases, and infections. Autoimmune myelofibrosis (AIMF) is an uncommon etiology of BMF; it can be secondary to a defined autoimmune disease, or it can be primary in the absence of a clinically diagnosed autoimmune disease but the presence of serologic evidence of autoantibodies. Distinguishing between primary myelofibrosis (PMF) and non-neoplastic AIMF is of the utmost im...

Autoimmune encephalitis following alemtuzumab treatment of multiple sclerosis.

Secondary autoimmune disorders are a recognised complication of alemtuzumab treatment for multiple sclerosis. We report a case of autoimmune encephalitis manifesting as a polymorphic epilepsia partialis continua / status epilepticus seven months after the second course of alemtuzumab in a patient with previous autoimmune hypothyroidism and immune thrombocytopenic purpura. An MRI revealed multifocal cortical abnormalities and neuronal loss was evident on biopsy. Although testing for anti-neuronal antibodies ...

A Case of Multiple Cerebral Infarction Preceding Acute Exacerbation of Idiopathic Thrombocytopenic Purpura.

Although it was suggested that idiopathic thromobocytopenic purpura (ITP) can be a paradoxical cause of cerebral infarction, previous reports indicate that cerebral infarction associated with ITP occurs when thrombocytopenia is already evident at the onset of cerebral infarction.

Risk Factors for Rate of Relapse and Effects of Steroid Maintenance Therapy in Patients with Autoimmune Pancreatitis: Systematic Review and Meta-analysis.

Risk for relapse after induction of remission with steroid therapy has been extensively studied in patients with autoimmune pancreatitis (AIP), but findings are equivocal. We performed a systematic review and meta-analysis were to estimate the rate of rate of AIP following initial remission after steroid treatment and to identify factors associated with relapse.

Chest pain with increased troponin level; not always a cardiology issue.

Thrombotic Thrombocytopenic Purpura (TTP) is a thrombotic microangiopathy syndrome resulting from decrease or absence of "a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13" (ADAMTS13). TTP has been characterized by the classical pentad of thrombocytopenia, hemolysis, fever, renal injury and neurological deficits, yet the patient may present with any atypical symptom related to microthrombi formation in the microcirculation. Here we present a rare case of a young patient with r...

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