Effect of a factor VIII concentrate on type IIB von Willebrand's disease-associated thrombocytopenia presenting during pregnancy in identical twin mothers

American Journal of Hematology
M IekoS Nakagawa

Abstract

Marked thrombocytopenia developed during pregnancy in both identical twins mothers who had systemic lupus erythematosus (SLE) and also type IIB von Willebrand's disease (vWD). The proband's platelet count decreased in the third trimester of pregnancy. Large-dose gamma-globulin and prednisolone treatments were performed because of the suspicion of immune thrombocytopenic reaction associated with SLE. These treatments were not effective. Her platelet count returned to the normal range immediately after delivery. Postpartum examinations revealed the decreased ristocetin cofactor activity and the deficiency of large von Willebrand factor (vWF) multimers in preserved plasma samples from the third trimester. These abnormal findings improved after delivery. Investigation of family members revealed that the proband had inherited type IIB vWD from her mother. The other twin, who was also under treatment for SLE, became pregnant about 1 year after delivery in the proband and followed almost the same course as that observed in the proband. As bleeding tendency was observed a few days before delivery, a factor VIII concentrate (Haemate P) was administered to compete with her variant vWF. This concentrate could prevent the further decrease ...Continue Reading

References

May 1, 1988·British Journal of Haematology·P A KyrleK Lechner
Apr 1, 1988·American Journal of Hematology·M F López-FernándezJ Batlle
Nov 1, 1987·British Journal of Haematology·A R GilesK Benford

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Citations

Jan 2, 2001·The Journal of Maternal-fetal Medicine·H RoquéC J Lockwood
Jan 5, 1999·Haemophilia : the Official Journal of the World Federation of Hemophilia·P A Kouides
Jul 1, 1997·Haemophilia : the Official Journal of the World Federation of Hemophilia
Jul 30, 1999·British Journal of Obstetrics and Gynaecology·D L EconomidesC A Lee
Jan 27, 2005·Journal of Thrombosis and Haemostasis : JTH·J L Kujovich
Feb 27, 2021·Seminars in Thrombosis and Hemostasis·Mona M MakhamrehHuda B Al-Kouatly
Sep 27, 2000·Hematology/oncology Clinics of North America·F A Nizzi, G Mues

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