Biomaterial Database

[Thrombotic microangiopathy following stem cell transplantation]. 2004

K Kentouche

Klinik für Kinder- und Jugendmedizin, Klinikum der Friedrich Schiller Universität, Kochstr. 2, 07743 Jena. Karim.Kentouche@med.uni-jena.de

Stem cell transplantation associated microangiopathy (TA-TMA) occurs in about 7% of all transplantations. While it resembles in all its features idiopathic TTP or HUS, response to plasma therapy is poor. The overall mortality of patients afflicted with TA-TMA is about 80%. Comparative studies aimed at discovering risk factors for the development of TA-TMA and defined different entities according to clinical, laboratory and outcome parameters. It is thought to be of multifactorial aetiology with the central event of endothelial damage. Intensive chemotherapy and radiation for bmt-preparation, infection in the immunocompromised host and graft versus host disease in conjunction with immunosuppressive therapy i. e. cyclosporine turn the haemostatic balance into a procoagulant state. Therapeutic strategies deduced from idiopathic TTP were disappointing with the only consensus in the withdrawal of cyclosporine from TA-TMA patients. Future trials are directed to ameliorate endothelial dysfunction.

UI	MeSH Term	Description	Entries
D008833	Microcirculation	The circulation of the BLOOD through the MICROVASCULAR NETWORK.	Microvascular Blood Flow,Microvascular Circulation,Blood Flow, Microvascular,Circulation, Microvascular,Flow, Microvascular Blood,Microvascular Blood Flows,Microvascular Circulations
D011697	Purpura, Thrombotic Thrombocytopenic	An acquired, congenital, or familial disorder caused by PLATELET AGGREGATION with THROMBOSIS in terminal arterioles and capillaries. Clinical features include THROMBOCYTOPENIA; HEMOLYTIC ANEMIA; AZOTEMIA; FEVER; and thrombotic microangiopathy. The classical form also includes neurological symptoms and end-organ damage, such as RENAL FAILURE. Mutations in the ADAMTS13 PROTEIN gene have been identified in familial cases.	Moschkowitz Disease,Purpura, Thrombotic Thrombopenic,Thrombotic Thrombocytopenic Purpura, Congenital,Thrombotic Thrombocytopenic Purpura, Familial,Congenital Thrombotic Thrombocytopenic Purpura,Familial Thrombotic Thrombocytopenia Purpura,Familial Thrombotic Thrombocytopenic Purpura,Microangiopathic Hemolytic Anemia, Congenital,Moschcowitz Disease,Schulman-Upshaw Syndrome,Thrombotic Microangiopathy, Familial,Thrombotic Thrombocytopenic Purpura,Upshaw Factor, Deficiency of,Upshaw-Schulman Syndrome,Familial Thrombotic Microangiopathy,Microangiopathy, Familial Thrombotic,Schulman Upshaw Syndrome,Thrombocytopenic Purpura, Thrombotic,Thrombopenic Purpura, Thrombotic,Thrombotic Thrombopenic Purpura,Upshaw Schulman Syndrome
D003937	Diagnosis, Differential	Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures.	Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D033581	Stem Cell Transplantation	The transfer of STEM CELLS from one individual to another within the same species (TRANSPLANTATION, HOMOLOGOUS) or between species (XENOTRANSPLANTATION), or transfer within the same individual (TRANSPLANTATION, AUTOLOGOUS). The source and location of the stem cells determines their potency or pluripotency to differentiate into various cell types.	Transplantation, Stem Cell,Stem Cell Transplantations,Transplantations, Stem Cell