Current management of thrombotic thrombocytopenic purpura

Kremer Hovinga, Johanna A; Meyer, Sara C (2008). Current management of thrombotic thrombocytopenic purpura. Current opinion in hematology, 15(5), pp. 445-50. Hagerstown, Md.: Wolters Kluwer Lippincott Williams & Wilkins 10.1097/MOH.0b013e328309ec62

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PURPOSE OF REVIEW: New treatment modalities have become increasingly popular for the treatment of acute thrombotic thrombocytopenic purpura. Widespread availability of ADAMTS13 assays resulted in the increased recognition of patients with hereditary thrombotic thrombocytopenic purpura and specific issues related to acquired ADAMTS13 deficiency. These new aspects with implications on management of thrombotic thrombocytopenic purpura patients are reviewed here. RECENT FINDINGS: Today, plasma exchange with the replacement of fresh frozen plasma is still the treatment of choice in acute thrombotic thrombocytopenic purpura. The finding of circulating anti-ADAMTS13 autoantibodies in the majority of patients constitutes the rationale for the concomitant administration of immunosuppressive drugs. Rituximab seems to have a favorable benefit-risk ratio in plasma-refractory and relapsing thrombotic thrombocytopenic purpura; however, long-term follow-up data are not yet available. Constitutively lacking ADAMTS13 in hereditary thrombotic thrombocytopenic purpura can be supplemented by simple plasma infusions. Severe acquired ADAMTS13 deficiency either at presentation or in remission identifies patients at a particularly high risk of relapse. SUMMARY: Despite progress in understanding the pathophysiology of thrombotic thrombocytopenic purpura, acute bouts as well as relapses still represent serious health threats to patients and rapid initiation of plasma exchange is mandatory. Large randomized clinical trials, however, need to determine whether new treatment modalities are superior to standard plasma exchange.

Item Type:

Journal Article (Further Contribution)


04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Haematology and Central Haematological Laboratory

UniBE Contributor:

Kremer Hovinga, Johanna Anna and Meyer, Sara Christina






Wolters Kluwer Lippincott Williams & Wilkins




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Date Deposited:

04 Oct 2013 15:02

Last Modified:

04 May 2014 23:18

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URI: (FactScience: 97205)

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