A 70 year old woman with no personal or family history of bleeding diathesis presented to emergency room with month long history of intermittent epistaxis. Her initial laboratory investigation showed severe macrocytic anemia and new onset renal failure. A presumptive diagnosis of uremic platelet dysfunction was made and the patient treated with anterior nasal packing and DDAVP. However, the patient continued to have bleeding even with anterior nasal packing. Her coagulation studies showed: APTT 89.3 seconds, PT 15.7 seconds and Platelet 246,000/dL. Inhibitor screening by mixing study was suggestive of the presence of circulating inhibitors; Factor VIII and IX levels were 275% & 223% of reference range respectively. Factor VIII inhibitor assay was positive for circulating inhibitors. Further work-up for anemia and renal failure revealed lambda monoclonal protein in the patient’s serum and urine. Skeletal survey showed impressive lytic lesions within the skull and other bones. A diagnosis of multiple myeloma was made.
Acquired hemophilia is a rare auto-immune disorder involving formation of inhibitory antibodies against factor VIII. The annual incidence varies between 1 to 1.5 people per million. These autoantibodies are associated with high rate of morbidity & mortality as severe bleeding occurs in up to 90% of affected patients with mortality rates between 8 to 22%. Although around 10% of the cases of acquired hemophilia are secondary to solid malignancies, the association between multiple myeloma and acquired hemophilia is extremely uncommon. Our patient’s presentation of new onset bleeding diathesis associated with presence of circulatory factor VIII inhibitor is highly suggestive of newly acquired hemophilia secondary to multiple myeloma. Factor VIII levels are low in patients with acquired hemophilia, but in our patient it was high, which was likely secondary to use of DDAVP.
In patients presenting with severe or refractory bleeding, autoantibodies against Factor VIII should be considered in the differential diagnosis and those diagnosed with acquired hemophilia, underlying etiology such as autoimmune disorder or malignancy should be sought.