Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies

Abstract

The guideline group was selected to be representative of UKbased medical experts. MEDLINE and EMBASE were searched systematically for publications in English, using the keywords: thrombotic thrombocytopenia purpura (TTP), AD-AMTS13, plasma exchange (PEX) and relevant key words related to the subsections of this guideline. The writing group produced the draft guideline, which was subsequently revised by consensus by members of the Haemostasis and Thrombosis Task Force of the BCSH. The guideline was then reviewed by a sounding board of British haematologists, the BCSH and the British Society for Haematology

Drug Induced Thrombotic Microangiopathy Caused by Levofloxacin

“…By day 9 of levofl oxacin, haemoglobin was 72 g/L and platelet count was 52 x 10 9 /L. Coagulation profi le was normal 1 Specialty Trainee, 2 (Table 1).…”

Egészségügyi szakmai irányelv – A thromboticus thrombocytopeniás purpura (TTP) és a haemolyticus uraemiás syndroma (HUS) kezeléséről

“…Amennyiben az adaptált irányelvek eltérő besorolási rendszert használtak, a hazai fejlesztőcsoport a BCSH irányelv [10] besorolási rendszerét vette át és az egyéb adaptált irányelvből származó ajánlásokat is ennek alapján sorolták be. Amennyiben az adaptált irányelvek egyegy ajánlásra eltérő fokozatot állapítanak meg, a fejlesztőcsoport az alacsonyabb fokozatú ajánlásbesorolást alkalmazza.…”

“…Az ADAMTS13 gén homozy gota vagy compound heterozygota mutációjának igazolásával a diagnózis megerősíthető (1A) [10].…”

Caplacizumab for Acquired Thrombotic Thrombocytopenic Purpura

“…1 Acquired TTP is caused by a severe deficiency of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) due to the presence of inhibitory autoantibodies. 2 Decreased ADAMTS13 activity leads to an accumulation of ultralarge von Willebrand factor multimers, which bind to platelets and induce aggregation.…”

“…[6][7][8][9][10] Treatment of acquired TTP consists of rapid initiation of plasma exchange to remove autoantibodies and ultralarge von Willebrand factor multimers and to replenish ADAMTS13. Immunosuppressive therapy (e.g., glucocorticoids and rituximab) 1,11 inhibits autoantibody formation. Although the survival rate among patients with acquired TTP exceeds 80%, 12 patients remain at risk for microthrombotic complications until remission is achieved.…”