Thrombotic thrombocytopenic purpura: medical and biological monitoring of six pregnancies

Background: Thrombotic thrombocytopenic purpura (TTP) is a rare cause of severe thrombocytopenia in pregnancy. Methods: Six pregnancies in five patients with TTP were followed prospectively over 5 years. Ultralarge von Willebrand factor (ULvWF) multimers and cleaving protease (cp) levels were measur...

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Published in:European journal of obstetrics & gynecology and reproductive biology 2003-12, Vol.111 (2), p.146-152
Main Authors: Ducloy-Bouthors, Anne-Sophie, Caron, Claudine, Subtil, Damien, Provot, François, Tournoys, Antoine, Wibau, Bénédicte, Krivosic-Horber, Renée
Format: Article
Language:English
Subjects:
Adult
Analgesia, Obstetrical
Biological and medical sciences
Cleaving protease
Diseases of mother, fetus and pregnancy
Female
Gestational Age
Gynecology. Andrology. Obstetrics
Hematologic and hematopoietic diseases
Humans
Medical sciences
Plasmapheresis
Platelet diseases and coagulopathies
Pregnancy
Pregnancy Complications, Hematologic
Pregnancy. Fetus. Placenta
Prospective Studies
Purpura, Thrombotic Thrombocytopenic - complications
Purpura, Thrombotic Thrombocytopenic - diagnosis
Purpura, Thrombotic Thrombocytopenic - therapy
Recurrence
Thrombocytopenia
Thrombotic thrombocytopenic purpura
von Willebrand Factor - analysis
von Willebrand Factor - chemistry
von Willebrand factor multimers
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Summary:Background: Thrombotic thrombocytopenic purpura (TTP) is a rare cause of severe thrombocytopenia in pregnancy. Methods: Six pregnancies in five patients with TTP were followed prospectively over 5 years. Ultralarge von Willebrand factor (ULvWF) multimers and cleaving protease (cp) levels were measured. Results: TTP relapsed, complicating four of the six pregnancies. Of three patients who relapsed, two had complete or partial vWF-cleaving protease (vWF-cp) deficiency, and one had a normal vWF-cleaving protease level. In all three we found abnormal UL multimers. The two women who did not relapse had normal vWF-cleaving protease level and an absence or loss of UL multimers. Conclusions: Pregnant patients with a history of TTP must be followed in a tertiary obstetric unit with plasmapheresis available. Influence of vWF-cleaving protease and vWF multimeric abnormalities on TTP relapsing during pregnancy has to be evaluated in a further multicentre study.
ISSN:0301-2115
1872-7654
DOI:10.1016/S0301-2115(03)00199-4