Management of Type 2B von Willebrand Disease during Pregnancy

Abstract

Type 2B von Willebrand disease is a rare bleeding condition resulting in thrombocytopenia and a reduction in large VWF multimers. It usually has an autosomal dominant pattern of inheritance. We report the management of a patient with type 2B von Willebrand disease, whose diagnosis was confirmed by demonstration of a R1306W mutation, through her first pregnancy. The patient's von Willebrand factor (VWF) antigen and VWF ristocetin cofactor levels rose throughout pregnancy, with an associated drop in the platelet count. The patient was successfully managed through labour to a surgical delivery with VWF concentrate, platelet transfusions and tranexamic acid. The patient delivered a male baby who was found to have inherited type 2B von Willebrand disease and had a significant cephalhaematoma at delivery. The baby was managed with VWF concentrate and platelet transfusions and made a full recovery. There is a lack of evidence to guide the best management of pregnant patients with type 2B von Willebrand disease. We adopted a pragmatic management plan, in keeping with other published case reports. To the best of our knowledge, this is the first case report in which the child was found to have inherited type 2B von Willebrand disease and encountered bleeding problems, making this case unique amongst the published literature.

Type 2B von Willebrand Disease: Early Manifestation as Neonatal Thrombocytopenia

“…So far, only single reports have been published describing diagnosis and therapy in newborns with VWD 2B. [8][9][10][11][12] We show that the course of severe thrombocytopenia can be pronounced depending on the kind of VWF mutation and on stressful events (i.e., intravenous blood drawing). In addition, we describe that treatment with VWF and, when needed, platelet concentrate transfusions prevented bleeding complications in this patient.…”

“…7 There are only few case reports of newborns affected by VWD 2B. [8][9][10][11][12] Premature newborns with VWD 2B may be at increased risk of hemorrhagic complications (i.e., intracerebral bleeding). Therefore, we continued treatment with VWF-containing FVIII (20-50 IE/kg body weight daily) (►Fig.…”

“…Literature concerning management of VWD 2B in the neonatal period is restricted to few case reports and expert opinions. [8][9][10][11][12]18 In all but one cases reported in the literature, the mothers of the affected newborns had already been known to suffer from VWD 2B. [8][9][10][11]18 Therefore, blood was withdrawn immediately after birth, revealing severe thrombocytopenia between 4 and 19 G/L.…”

“…[8][9][10][11][12]18 In all but one cases reported in the literature, the mothers of the affected newborns had already been known to suffer from VWD 2B. [8][9][10][11]18 Therefore, blood was withdrawn immediately after birth, revealing severe thrombocytopenia between 4 and 19 G/L. [8][9][10][11] All newborns from mothers with a known VWD 2B received VWF-containing blood products and platelet transfusions directly after birth.…”

Report on the management of thrombocytopenia in obstetric patients: A retrospective study

“…The study by McLaughlin and Kerr [ 15 ] reported that there is a loss of evidence or document to guide the best management of pregnant patients with thrombocytopenia disease.…”