Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura.
Details
Serval ID
serval:BIB_E11A4CDDCA61
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura.
Journal
Haematologica
ISSN
1592-8721 (Electronic)
ISSN-L
0390-6078
Publication state
Published
Issued date
2004
Peer-reviewed
Oui
Volume
89
Number
3
Pages
320-324
Language
english
Notes
Publication types: Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't Publication Status: ppublish
Abstract
BACKGROUND AND OBJECTIVES: Acquired thrombotic thrombocytopenic purpura (TTP) is often due to autoantibodies inhibiting ADAMTS-13 activity resulting in impaired processing of very large von Willebrand factor multimers. TTP usually presents with an acute onset and a fulminant, sometimes fatal course. With appropriate treatment including plasma exchange, and fresh frozen plasma replacement, often supplemented by immuno-suppressive therapy, the acute episode generally resolves within days to weeks.
DESIGN AND METHODS: We describe the clinical course of 3 patients with acquired TTP. One was refractory to PE, the other 2 relapsed after this treatment. All three were treated with splenectomy. ADAMTS-13 activity and inhibitor levels were monitored.
RESULTS: ADAMTS-13 activity was initially < 5% in all 3 patients. After splenectomy the inhibitor against ADAMTS-13 disappeared rapidly in 2 patients and there was full recovery of ADAMTS-13 activity in all 3 patients.
INTERPRETATION AND CONCLUSIONS: Splenectomy, by eliminating a source of pathogenic autoantibody production, can be a successful treatment for patients with relapsing or plasma-refractory acquired TTP due to autoantibody-mediated ADAMTS-13 deficiency.
DESIGN AND METHODS: We describe the clinical course of 3 patients with acquired TTP. One was refractory to PE, the other 2 relapsed after this treatment. All three were treated with splenectomy. ADAMTS-13 activity and inhibitor levels were monitored.
RESULTS: ADAMTS-13 activity was initially < 5% in all 3 patients. After splenectomy the inhibitor against ADAMTS-13 disappeared rapidly in 2 patients and there was full recovery of ADAMTS-13 activity in all 3 patients.
INTERPRETATION AND CONCLUSIONS: Splenectomy, by eliminating a source of pathogenic autoantibody production, can be a successful treatment for patients with relapsing or plasma-refractory acquired TTP due to autoantibody-mediated ADAMTS-13 deficiency.
Keywords
Adolescent, Adult, Aged, Autoantibodies/immunology, Female, Follow-Up Studies, Humans, Male, Metalloendopeptidases/antagonists & inhibitors, Metalloendopeptidases/immunology, Plasma Exchange, Purpura, Thrombotic Thrombocytopenic/etiology, Purpura, Thrombotic Thrombocytopenic/immunology, Splenectomy
Pubmed
Web of science
Create date
09/11/2014 17:32
Last modification date
20/08/2019 16:05