Universität zu Köln

Voelker, Linus A., Brinkkoetter, Paul T., Knoebl, Paul N., Krstic, Miroslav, Kaufeld, Jessica, Menne, Jan, Buxhofer-Ausch, Veronika and Miesbach, Wolfgang (2020). Treatment of acquired thrombotic thrombocytopenic purpura without plasma exchange in selected patients under caplacizumab. J. Thromb. Haemost., 18 (11). S. 3061 - 3067. HOBOKEN: WILEY. ISSN 1538-7836

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Abstract

Background Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening autoimmune thrombotic microangiopathy. Current standard of care is therapeutic plasma exchange, immunosuppression, and caplacizumab, an anti-von Willebrand factor nanobody, which is effective in treating aTTP episodes. Patients/Methods Here we report on seven episodes of aTTP treated without plasma exchange in six female patients in Germany and Austria. Two episodes were initial presentations of aTTP; in five instances, patients experienced a relapse. In four episodes, moderate to severe organ dysfunction was observed; three cases presented with a mild course. All patients received caplacizumab immediately once aTTP was suspected or diagnosed, and plasma exchange was omitted based on shared decision making between patient and the treating physicians. Results We observed a rapid and robust increase of platelet counts already after the first dose of caplacizumab, leading to a doubling of platelet counts within 17 hours (median), platelet counts normalized (>150 G/L) after median 84 hours. Lactate dehydrogenase, as a surrogate parameter of organ damage, improved in parallel to the platelet counts, indicating resolving microangiopathy. Conclusions In conclusion, in selected cases of acute bouts of aTTP, it seems feasible to delay or omit plasma exchange if platelet counts increase and organ function is stable after start of caplacizumab therapy.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Voelker, Linus A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Brinkkoetter, Paul T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Knoebl, Paul N. UNSPECIFIED UNSPECIFIED UNSPECIFIED Krstic, Miroslav UNSPECIFIED UNSPECIFIED UNSPECIFIED Kaufeld, Jessica UNSPECIFIED UNSPECIFIED UNSPECIFIED Menne, Jan UNSPECIFIED UNSPECIFIED UNSPECIFIED Buxhofer-Ausch, Veronika UNSPECIFIED UNSPECIFIED UNSPECIFIED Miesbach, Wolfgang UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-319660
DOI:	10.1111/jth.15045
Journal or Publication Title:	J. Thromb. Haemost.
Volume:	18
Number:	11
Page Range:	S. 3061 - 3067
Date:	2020
Publisher:	WILEY
Place of Publication:	HOBOKEN
ISSN:	1538-7836
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language COMPLICATIONS Multiple languages Hematology; Peripheral Vascular Disease Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/31966