α1-antitrypsin therapy in a case of thrombotic thrombocytopenic purpura

M. Galbusera, P. Ruggenenti, M. Noris, A. Benigni, G. Remuzzi, P. Ruggenenti, G. Remuzzi, M. Burnouf-Radosevich, P. M. Mannucci

Research output: Contribution to journalArticlepeer-review

Abstract

In thrombotic thrombocytopenic purpura (TTP), activated leucocytes release elastase which mobilises unusually large von Willebrand factor (vWF) multimers from the endothelium. We investigated the effect of an inhibitor of leucocyte elastase (α1-antitrypsin) on circulating vWF and platelet count in a patient with chronic relapsing TTP. α1-antitrypsin consistently normalised vWF multimeric composition but failed to increase platelet count and induce remission. Plasma cryosupernatant, which never normalised vWF, always induced laboratory and clinical remission within 96 hours. In chronic relapsing TTP, unusually large vWF multimers are not the main cause of intravascular platelet aggregation.

Original languageEnglish
Pages (from-to)224-225
Number of pages2
JournalLancet
Volume345
Issue number8944
DOIs
Publication statusPublished - Jan 28 1995

ASJC Scopus subject areas

  • Medicine(all)

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