Publication

Abstract : A 63 years male presented with a history of recurrent episodes of lower gastrointestinal bleeding for three years. He had also a history of occasional epistaxis and skin bleeds for last 3 months. Repeated endoscopic gastrointestinal examinations demonstrated the presence of multiple angiodysplastic lesions in the descending and sigmoid colon. Family history revealed no members with known bleeding disorders. On examination, he was pale and there were few scattered generalized petechiae. Other physical findings were unremarkable. Hemogram showed Hb of 7.4gm /dl, TLC of 6.4 × 109 / L, platelet count of 230 × 109 / L. Peripheral smear examination showed microcytic hypochromic anemia with no abnormal cells. Initial coagulation screening revealed a normal prothrombin time (PT); a prolonged activated partial thromboplastin time (APTT) and a prolonged Ivy bleeding time. At presentation, FVIII procoagulant activity (20%), vWF antigen levels (22%), and ristocetin cofactor activity (13%) were markedly reduced. The ratio of VWF:RCo : VWF:Ag was less than 0.7. Multimeric analysis revealed loss of high molecular weight (HMWM) and intermediate molecular weight multimers. An in-vitro Inhibitor against vWF of the patientÊs plasma was demonstrated. A final diagnosis of AVWD subtype 2A with inhibitors to VWF was made. The patientÊs bleeding episodes were initially managed with cryoprecipitate replacement and desmopressin therapy to which he became refractory. Patient was subsequently started on steroid therapy (60 mg/day) as a measure of immunosupression. He continued to have bleeding episodes without any significant improvement. Patient was then managed with plasmapheresis followed by intravenous immunoglobulins (20g/day for 5 days) to which he showed an adequate response in terms of his clinical situation and his haemostatic parameters. After 2 months patient was planned for hemicolectomy, but before surgery he had massive inferior myocardial infarction and died.