Successful management of a bleeding diathesis in a patient with an acquired factor v inhibitor using platelet transfusions and mmunosuppressive therapy
Clinical Trials as Topic
A 75 year old male with pernicious anemia presented with gross hematuria, hemoptysis, and rectal bleeding. Laboratory evaluation revealed PT >150 sees, INR>50, APTT >150 sees. Thrombin time was normal. Factor II,VII,VIII, X and fibrinogen levels were all normal. However, Factor V level was <0.01 U/ml. DRVVT was negative. Mixing studies with incubation revealed prolongation of both the PT and APTT. Factor V inhibitor was confirmed by the Bethesda method with titre of 4.2BU. Prior to our consultation, infusion of 12 units of fresh fozen plasma resulted in improvement of neither laboratory nor clinical parameters. Single donor platelet transfusions were instituted every 12 hours and continued for 12 days. Simultaneously, immunosuppression with prednisone (lmg/kg/day) plus cyclophosphamide (lOOrng) daily by mouth was begun. By day 11, bleeding had stopped, PT had decreased to 18.1 secs, INR to 1.59, APTT to 44 sees, and Factor V levels had increased to 58 U/ml with Bethesda units absent. He had received a total of 6 units of packed red blood cells. Cytoxan and prednisone continue to be gradually tapered. 105 cases of Factor V inhibitor have been published between 1955 and 1997. Its occurrence has been associated with the post-operative state, thrombin and fibrin glue, drugs such as beta-lactam antibiotics and aminoglycosides, malignancy, and rarely the post-partum state. Underlying autoimmune disorders such as Sjogren's syndrome.celiac disease, and bullous pemphigoid have been associated in a small number of cases. Our patient was found to have pernicious anemia with associated anti-intrinsic factor antibodies three months prior to presentation. We suspect a significant association between his autoimmune disorder and development of a Factor V inhibitor. His bleeding diathesis responded dramatically to immunosuppressive therapy and platelet transfusions. 50:50 mix immediate 1 hr 2 his APTT(Patient) 73.5 sees .150 secs 148.7 sees APTT(Control) 31.7 sees 31.5 sees 32.2 sees PT (Patient) 33.4 sees 53.2 sees 62.7 sees PT(Control) B.lsecs 13.3 sees 13.8 sees.