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Acquired von Willebrand's syndrome associated with an extranodal pulmonary lymphoma
1Department of Pathology and Laboratory Medicine, Medical University of South Carolina, Charleston 29425.
A case of acquired von Willebrand's syndrome associated with an extranodal pulmonary lymphoma is reported in a 58-year-old man. His initial factor VIII-von Willebrand factor (vWF) complex parameters included a factor VIII activity of 29 U/dL, a vWF protein of 17 U/dL, and a ristocetin cofactor of less than 10 U/dL. A specific factor VIII inhibitor could not be demonstrated in mixtures of his plasma and normal pooled plasma nor could immune complexes of IgG-factor VIII be detected in similar mixtures using protein A in a solid phase. Following surgical removal of the patient's tumor, all factor VIII-vWF complex parameters returned to normal. Immunoperoxidase stains of the lymphoid tumor cells were negative for von Willebrand protein. The patient's acquired von Willebrand's syndrome recurred approximately one year later, presumably indicative of recurrent lymphoma.
A case of acquired von Willebrand's syndrome associated with an extranodal pulmonary lymphoma is reported in a 58-year-old man. His initial factor VIII-von Willebrand factor (vWF) complex parameters included a factor VIII activity of 29 U/dL, a vWF protein of 17 U/dL, and a ristocetin cofactor of less than 10 U/dL. A specific factor VIII inhibitor could not be demonstrated in mixtures of his plasma and normal pooled plasma nor could immune complexes of IgG-factor VIII be detected in similar mixtures using protein A in a solid phase. Following surgical removal of the patient's tumor, all factor VIII-vWF complex parameters returned to normal. Immunoperoxidase stains of the lymphoid tumor cells were negative for von Willebrand protein. The patient's acquired von Willebrand's syndrome recurred approximately one year later, presumably indicative of recurrent lymphoma.