ZQ Zhang, GCF Chan, CCK Lam, JCC So, DKL Cheuk, AKS Chiang, SY Ha

Abstract

The information related to the clinical spectrum of von Willebrand disease (VWD) in Chinese patients remains very limited. We conducted a retrospective chart review on the clinical and haematological features of VWD among Chinese patients at a tertiary paediatric centre in Hong Kong. Ten patients (6 females, 4 males) were diagnosed to have VWD from 1989 to 2005. They underwent treatment in our unit, with a cumulative follow up of 102 patient-years within this 16-year period. Among them, 4 were type 1, 5 were type 2 and 1 was type 3 VWD. Six of the 10 patients had a positive family history of bleeding tendencies. A variety of bleeding manifestations were observed in these patients while mucocutaneous bleeds in the form of frequent epistaxis and easy bruising were the commonest presenting features. Severe bleeding in the form of intracranial haemorrhage occurred in 2 patients. Eight patients underwent desmopressin (DDAVP) test at diagnosis and all were responsive to DDAVP without associated thrombocytopenia. Three patients required frequent DDAVP (intravenous or subcutaneous) and 2 required occasional intermediate purity factor VIII concentrate for bleeding control. In conclusion, majority of Chinese paediatric VWD patients are inherited and acquired form is extremely rare in childhood. Patients with either type 1 or 2 VWD can develop severe bleeding in childhood. In our patient cohort, DDAVP appears to be effective and safe for our patients with either type 1 VWD or non-2B type 2 VWD without inducing thrombocytopenia.