The thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are acute, fulminant extending diseases which are characterized by thrombocytopenia and microangiopathic hemolytic anemia. Additional clinical manifestations may include impaired consciousness and kidney failure. For the diagnosis characteristic laboratory abnormalities, including evidence of a Coombs-negative hemolytic anemia, necessary. The treatment consists in the plasma exchange and corticosteroids in adults and is supportive (possibly hemodialysis) in children; Eculizumab is rarely indicated.

The thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are acute, fulminant extending diseases which are characterized by thrombocytopenia and microangiopathic hemolytic anemia. Additional clinical manifestations may include impaired consciousness and kidney failure. For the diagnosis characteristic laboratory abnormalities, including evidence of a Coombs-negative hemolytic anemia, necessary. The treatment consists in the plasma exchange and corticosteroids in adults and is supportive (possibly hemodialysis) in children; Eculizumab is rarely indicated. Pathophysiology TTP and HUS it comes to non-immunological reduction of platelets. Free platelets and Fibrinfasen deposited in numerous small vessels and damage flowing past platelets and red blood cells, resulting in a significant thrombocytopenia and anemia. Platelets also aggregate into multiple small thrombi. In many organs thrombi of platelets and von Willebrand factor, however, develop without granulocytic infiltration of blood vessel walls, as is characteristic of a vasculitis. These blood clots are mainly located in the arteriokapill√§ren stream bed. Therefore, the disease is also known as thrombotic microangiopathy. Brain, heart and kidneys are particularly affected. TTP and HUS mainly differ in the severity of renal insufficiency. In adults TTP is mainly described, renal failure rarely occurs sooner. By contrast, it comes in children mostly for HUS, typically with kidney failure. Etiology children producing by Shiga toxin bacteria (eg, Escherichia coli O157:. H7, some strains of Shigella dysenteriae- infection by Escherichia coli O157: H7 and other enterohemorrhagic E. coli (EHEC)) there is usually an acute hemorrhagic colitis .Erwachsene Many cases are idiopathic. Among the known causes and circumstances immunosuppressants (. Eg cyclosporine), and chemotherapeutic agents (often indistinguishable from a severe pre-eclampsia or eclampsia) include (eg mitomycin C.) Pregnancy Hemorrhagic colitis caused by Escherichia coli O157: H7 or E. coli O104: H4 In many patients, there is a predisposition by a congenital or acquired deficiency of the plasma enzyme ADAMTS13. The protease cleaves the von Willebrand factor and thus eliminates abnormally large von Willebrand factor multimers leading to thrombus formation. Symptoms and complaints of multiple characters Organisch√§mien develop in varying degrees of severity. For this weakness, confusion or coma, abdominal pain, nausea, vomiting, diarrhea, as well as arrhythmias myocardial damage can include. Children usually have a prodromal stage with vomiting, abdominal pain and (often bloody) diarrhea. usually not fever occurs in TTP or HUS. The symptoms and discomfort of TTP and HUS are indistinguishable, except that neurological symptoms occur rarely with HUS. Diagnostic blood count with platelets, peripheral blood smear, Coombs test, LDH, PT, PTT, fibrinogen exclusion of other thrombocytopenic diseases Suspected TTP-HUS, patients with suspicious symptoms, thrombocytopenia and anemia. In these cases, a urine analysis, peripheral blood smears, reticulocyte count, serum LDH, retention parameters, serum bilirubin should be carried out (direct and indirect) and Coombs test. Diagnosis-setting are thrombocytopenia and anemia fragmented erythrocytes (fragmentocytes) in the blood smear indicate a microangiopathic hemolysis (schistocytes: helmet shapes, triangular shaped erythrocytes, torn appearing erythrocytes) towards signs of hemolysis (decreasing hemoglobin levels, polychromasia, increased reticulocyte count, increased serum LDH and bilirubin, reduced haptoglobin) negative direct Coombs test Unexplained thrombocytopenia and microangiopathic hemolytic anemia are a sufficient proof to make this diagnosis. Schistocytes (erythrocyte fragments) with permission of the publisher. From Tefferi A., Li C. In Atlas of Clinical Hematology. Edited by J. O. Armitage. Philadelphia, Current Medicine, 2004. var model = {thumbnailUrl: ‘/-/media/manual/professional/images/schistocytes_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/ – / media / manual / professional / images / schistocytes_high_de.jpg lang = en & thn = 0 ‘, title:’? schistocytes (erythrocyte fragments) ‘description:’ u003Ca id = “v38396074 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “”para “” u003e u003cp u003eSchistozyten (arrows) are destroyed erythrocytes in microangiopathic anemia (including disseminated intravascular coagulation

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