Thrombotic microangiopathy (TMA) is a histopathological feature of various diseases including thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). There are many secondary causes of TMA, many of them could mimic TTP or HUS. This article presents a short overview on TMA. In conclusion TMA is the result of various etiology reasons and pathologic reactions with various clinical entities. It is important to focus on a thorough history including family history when deciding on a diagnosis. Analysis of ADAMTS 13 and ADAMTS 13-antibodies may help to decide continued therapy.
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http://dx.doi.org/10.1016/j.transci.2011.07.002 | DOI Listing |
Kidney Int
January 2025
Complement Therapeutics Research Group, Newcastle University Translational and Clinical Research Institute, The Medical School, Newcastle-upon-Tyne, UK; National Renal Complement Therapeutics Centre, The Royal Victoria Infirmary, Newcastle-upon-Tyne, UK.
Introduction of complement (C) inhibition into clinical practice has revolutionized the treatment of patients with complement-mediated atypical hemolytic syndrome (aHUS). Our C3 mouse model, engineered around a gain of function point mutation in C3, is associated with complement mediated aHUS in man, allowing us to study the clinical disease in a preclinical model. Backcrossing our model onto C7 deficient and C5aR1 deficient mice enabled further determination of the roles of the C5a-C5aR1 axis and C5b-9 (the membrane attack complex) on kidney disease.
View Article and Find Full Text PDFFront Med (Lausanne)
January 2025
Department of Hematology, The Second Affiliated Hospital of Nanchang University, Nanchang, China.
Congenital thrombotic thrombocytopenic purpura (cTTP) is a thrombotic microangiopathy (TMA) characterized by severe hereditary ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motifs 13) deficiency caused by mutations. This rare autosomal recessive genetic disorder is often misdiagnosed as immune thrombocytopenia (ITP) or hemolytic uremic syndrome (HUS). Here, we report a 21-year-old male cTTP patient with a compound heterozygous mutation.
View Article and Find Full Text PDFRev Inst Med Trop Sao Paulo
January 2025
Hospital Militar Central, Servicio de Infectología, Bogotá, Colombia.
Immune thrombocytopenia (ITP) is an autoimmune hematological condition characterized by a markedly isolated decrease in platelets without any apparent associated clinical conditions, resulting in bleeding and bruising of the skin, mucous membranes, and major organs. It is often triggered by preceding illness or several immune stimulants such as immunizations, infections, allergic reactions, among others. While uncommon, arthropod bites can trigger acute ITP.
View Article and Find Full Text PDFInn Med (Heidelb)
January 2025
MVZ Nephrocare Mettmann GmbH, Gartenstr. 4-8, 40822, Mettmann, Deutschland.
Recognizing anemia and thrombpenia in acute and emergency medicine is easy. Acute (microangiopathic hemolytic) anemia and thrombopenia can be a sign of thrombotic microangiopathy (TMA). TMA syndromes are potentially life-threatening diseases.
View Article and Find Full Text PDFFront Pharmacol
January 2025
Department of Fetal Medicine, The First Affiliated Hospital of Jinan University, Guangzhou, China.
Onasemnogene Abeparvovec (Zolgensma) is a gene therapy for the treatment of Spinal Muscular Atrophy (SMA) with improved motor neuron function and the potential for a singular treatment. Information on its adverse drug reactions is mainly from clinical trials and real-world studies with extensive sample sizes are lacking. In this study, we analyzed the U.
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