Hemostatic Defect due to Acquired Circulating Inhibitors against Lipid Procoagulant and Factor VIII^[*]

Summary

An 85 year old woman was studied because of severe bleeding. Acquired inhibitors to factor VIII and to phospholipid procoagulants were demonstrated. Platelet factor 3 (Pf3) assay was prolonged with both kaolin and Russell’s Viper Venom (Stypven^E). It was normal with patient’s washed platelets and normal plasma, but abnormal when normal platelets were incubated with patient’s plasma. The inibitor also blocked the coagulant action of Bell and Alton thromboplastin, inosithin, phosphatidyl ethanolamine and phosphatidyl choline, but not that of tissue thromboplastin or cardiolipin. All other platelet functions were normal. The inhibitors were purified by A1 (OH₃) absorption, heating at 56°, precipitation by 50% ammonium sulfate, followed by dialysis and DEAE-cellulose chromatography. A partial separation of the two inhibitors was achieved.

Cyclophosphamide treatment resulted in cessation of bleeding and disappearance of the inhibitors. This seems to be the first instance of an acquired circulating inhibitor specifically directed against phospholipid procoagulants in a patient who also had an inhibitor to Factor VIII.

^* Presented in part at the Third Congress of the International Society on Thrombosis and Haemostasis, August 22-26, 1972, Washington, D. C.

⁺ Present address: Hematology-Oncology Section, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania 19104.

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