Type 2B Von Willebrand Disease in Pregnancy: A Systematic Literature Review [36K

Abstract

INTRODUCTION: Type 2B Von Willebrand Disease (VWD) is a rare hereditary bleeding disorder that is associated with thrombocytopenia. Our objective was to review obstetric complications in women with Type 2B VWD. METHODS: A systematic literature search was conducted using PubMed, Scopus, and Cochrane Central Register of Controlled Trials to include all publications that address Type 2B VWD in pregnancy. Our primary and secondary outcomes were incidence of postpartum hemorrhage (PPH) and incidence of thrombocytopenia in pregnancy, respectively. RESULTS: 20 studies met inclusion criteria. There were 32 pregnancies with Type 2B VWD in 27 women. Primary PPH was reported in 9/20 (45%) and secondary PPH was reported in 6/13 (46%) and occurred between 5-32 days. Factor concentrate treatment was administered before delivery and postpartum in 16 and 18 pregnancies, respectively. 17 pregnancies required blood products postpartum. Platelet transfusion was given in 13/17 (76%) pregnancies. Red blood cell transfusion was performed in 6/17 (35%) pregnancies. Thrombocytopenia in pregnancy was reported in 27/28 (96%). 23/27 (85%) were severe thrombocytopenia with mean platelet count of 33.7±22.7 × 103/ul and 4/27 (15%) were mild thrombocytopenia (range, 126-150 × 103/ul). No maternal mortality was reported. CONCLUSION: Type 2B VWD in pregnancy has significant morbidity, with high incidence of primary and secondary PPH. The majority of patients required the use of factor concentrate before and after delivery in addition to postpartum blood products. The incidence of severe thrombocytopenia was also high, indicating that Type 2B VWD should be considered in the differential diagnosis of severe thrombocytopenia in pregnancy.