Thrombotic thrombocytopenic purpura

Bukowski, Martin J.; Koblenzer, Peter J.

doi:10.1016/s0022-3476(62)80013-4

Cited by 30 publications

(23 citation statements)

References 36 publications

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“…Some investigators have asserted that TTP is associated with pregnancy, autoimmune connective tissue disorders, neoplasm, organ transplants, and certain drugs. Nevertheless, a comprehensive review of the literature by Bukowski in 1982 did not find sufficient evidence in support of such an association (61). Surprisingly, our own experience in more than 300 cases of TTP patients with ADAMTS13 inhibitors generally supports the observations made by Bukowski more than 25 years ago.…”

Section: Etiologymentioning

confidence: 48%

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ADAMTS13 and microvascular thrombosis

Tsai¹

2006

Expert Review of Cardiovascular Therapy

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SummaryInteraction between platelet and von Willebrand factor (VWF), a circulating adhesive glycoprotein, is essential for hemostasis under the high shear environments of arterioles and capillaries. If unregulated, this interaction may lead to unwarranted platelet thrombosis ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif, number 13), a plasma zinc metalloprotease synthesized primarily in the stellate cells of the liver, cleaves shear stress activated VWF, thereby preventing the occurrence of VWF-platelet interaction in the circulation. A profound deficiency of ADAMTS13, due to genetic mutations or autoimmune inhibitors, results in intravascular VWFplatelet aggregation and widespread microvascular thrombosis characteristic of thrombotic thrombocytopenic purpura (TTP). Cloning of ADAMTS13 and structure-function analysis of the enzyme are leading to exciting advances in the diagnosis and therapy of this hitherto mysterious disease.

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Section: Etiologymentioning

confidence: 48%

“…Although corticosteroids are often used, the overall response rate was only 11 percent before plasma therapy was introduced (61). The dismal response rate of corticosteroids for TTP is not unexpected, in view of their ineffectiveness in decreasing the antibody levels in other conditions such as factor V or factor VIII inhibitors.…”

Section: Treatment Acquired Ttpmentioning

confidence: 99%

ADAMTS13 and microvascular thrombosis

Tsai¹

2006

Expert Review of Cardiovascular Therapy

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“…However, the titers are low and concurrent presentation of active lupus or other autoimmune diseases is uncommon with TTP [66]. ADAMTS13 activity level may be partially decreased in patients with systemic lupus erythematosus or related disorders [67].…”

Section: Diagnosismentioning

confidence: 99%

Thrombotic Thrombocytopenic Purpura: A Thrombotic Disorder Caused by ADAMTS13 Deficiency

Tsai

2007

Hematology/Oncology Clinics of North America

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“…TTP has been described in association with SLE (1,9,11). It has also been reported to occur during pregnancy, with a few patients having been successfully treated by plasma exchange or plasma infusion ( I ) .…”

mentioning

confidence: 99%