Search results for " Purpura"

Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management

2021

Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and ischemic end organ injury due to microvascular platelet-rich thrombi. TTP results from a severe deficiency of the specific von Willebrand factor (VWF)-cleaving protease, ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). ADAMTS13 deficiency is most commonly acquired due to anti-ADAMTS13 autoantibodies. It can also be inherited in the congenital form as a result of biallelic mutations in the ADAMTS13 gene. In adults, the condition is most often immune-mediated (iTTP) whereas congenital TTP (cTT…

Algoritmo diagnóstico-terapéutico de las microangiopatías trombóticas. A propósito de 2 casos

2022

Thrombotic microangiopathies (TMA) are a group of clinical syndromes associated with haemolytic anaemia, thrombocytopenia and organ dysfunction, mainly renal or neurological. They are associated with significant morbidity and mortality, so early diagnosis and treatment are essential. In this article we report two cases of TMA; a patient with thrombotic thrombocytopenic purpura (TTP) and a patient with atypical haemolytic uraemic syndrome (aHUS).

Thrombotic thrombocytopenic purpura (TTP) leading to pseudotumour's autoimmune pancreatitis (AIP): A case report

2012

International audience; Introduction: Autoimmune pancreatitis is an idiopathic inflammatory disease that produces pancreatic masses and ductal strictures. This benign disease can be associated with extrapancreatic manifestations including cholangitis, sialadenitis, inflammatory bowel disease or retroperitoneal fibrosis, mediastinal adenopathy, interstitial nephritis mainly due to immunoglobulin G4 (Ig G4), and occasional association with other auto-immune diseases. Observation: We report a 57-year-old woman who developed thrombotic thrombocytopenic purpura (UP) and pseudo-tumour's seronegative autoimmune pancreatitis (ATP) type 1. The patient was initially treated with pulse corticosteroids…

Patterns of changes of anti‐ADAMTS13 after plasma exchange

2006

An enzyme-linked immunosorbent assay (ELISA) has recently been developed to detect antibodies against the von Willebrand factor-cleaving protease ADAMTS13 in patients with thrombotic thrombocytopenic purpura (TTP). The ELISA is based on incubation of plasma with immobilized recombinant ADAMTS13 followed by visualization of IgG and IgM antibodies by means of secondary enzyme-labeled antibodies [1]. In a recent study, anti-ADAMTS13 IgG antibodies were detected in most patients with TTP (97%) characterized by severe ADAMTS13 deficiency (< 10% of normal) [1]. The ELISA was more sensitive than the standard inhibitor assay based upon ADAMTS13 neutralizing activity, which gave positive results in …

Relapsing or refractory idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: the role of rituximab.

2010

The Developmental Transcriptome for Lytechinus variegatus Exhibits Temporally Punctuated Gene Expression Changes

2019

AbstractEmbryonic development is arguably the most complex process an organism undergoes during its lifetime, and understanding this complexity is best approached with a systems-level perspective. The sea urchin has become a highly valuable model organism for understanding developmental specification, morphogenesis, and evolution. As a non-chordate deuterostome, the sea urchin occupies an important evolutionary niche between protostomes and vertebrates.Lytechinus variegatus(Lv) is an Atlantic species that has been well studied, and which has provided important insights into signal transduction, patterning, and morphogenetic changes during embryonic and larval development. The Pacific specie…