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

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 in her platelet count, thereby correcting the hemorrhagic tendency. It seems evident that factor VII concentrate would be effective in treating thrombocytopenia associated with type IIB vWD.

Experience with Haemate P in von Willebrand’s Disease in Adults

“…Reports of successful use of Hae mate P in vWD were also published by Scharrer and Vigh [5], Takahashi et al [6], Czapek et al [7], Fukui et al [1], Vigh et al [8], Berntorp and Nilsson [2], Ieko et al [9], Rose et al [10], Logan [11], Mannucci et al [12], Rodeghiero et al [13], Yoshioka et al [14], and Foster [15], Several other new FVIII concentrates look promising for treatment of vWD. Successful use of SD-FVIII concentrates and other FVIII preparations were reported by Furlan et al [16], Gazengel et al [ 17], Mazurier et al [18], Logan and Higgins [ 19], Pasi et al [20], Palmer et al [21], Cumming et al [22], Lawrie et al [23], Oates et al [24], Retzios et al [25], and Hanna et al [26].…”

von Willebrand disease and pregnancy

“…Thrombocytopenia may develop or worsen during pregnancy. Women with type 2B VWD may present with thrombocytopenia during pregnancy [28,46–50]. In several cases, thrombocytopenia occurred only during pregnancy, with normal platelet counts documented after delivery [28,46–48,50].…”

“…Women with type 2B VWD may present with thrombocytopenia during pregnancy [28,46–50]. In several cases, thrombocytopenia occurred only during pregnancy, with normal platelet counts documented after delivery [28,46–48,50]. The severity of thrombocytopenia varies, with nadir values occasionally as low as 10 000–20 000 mm −3 at term [20,46,47,49].…”

“…The severity of thrombocytopenia reflects both the amount of variant VWF produced and the bone marrow's ability to increase platelet production. The concentration of variant VWF may be high enough to cause thrombocytopenia only during pregnancy, explaining the frequent normalization of platelet counts postpartum [28,49,50]. The extent to which thrombocytopenia contributes to the bleeding risk is unknown but probably depends on the severity.…”

Treatment and management of Von Willebrand disease

“…In some cases [17] the development of thrombocytopenia does not appear to exert an additive effect on the underlying haemostatic defect, so that active intervention is not necessary. In others, in whom the platelet count falls to dangerous levels, the use of VWF concentrates can cause a resolution of the thrombocytopenia [18].…”