A 24-year-old patient with isolated IgA deficiency and a 3-year history of minimal change glomerulonephritis with nephrotic syndrome developed acute hemorrhagic diathesis. A spontaneous inhibitor of factor VIII was diagnosed. Therapy with substitution and plasmapheresis was without prolonged effect. Only consistent immunosuppressive therapy normalized coagulation. The patient died from septic complications during immunosuppressive therapy.
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