Acquired hemophilia presenting as gross hematuria following kidney stone - a case report and review of the literature

ABSTRACT A rare condition in itself, acquired hemophilia A, seldom presents as isolated gross hematuria. It is a serious condition with a high mortality rate and thus clinical suspicion followed by prompt diagnosis is imperative (1). In fact, only 8 cases of such presentation of this condition have been reported thus far in the literature. Of these, none describe the initial presentation of hematuria with the inciting event of a kidney stone. We present a case of a 67-year-old man with signs and symptoms of nephrolithiasis accompanied by profuse hematuria, who was subsequently found to have developed expression of factor VIII inhibitor leading to acquired hemophilia A.

Treatment of Bleeding in Hemophilia

The management of hemophilia patients includes the treatment of acute bleeding, prevention of bleeding, and the treatment of complications. Bleeding can be minimized by prophylactic factor maintenance, education, and lifestyle modifications. The complications in hemophilia include hemophilic arthropathy, inhibitor development, liver disease due to transfusion-transmitted infections, etc. Patients can be managed ideally in comprehensive hemophilia care centers. The management of bleeding is most important. Treatment should be given early with an appropriate amount of drugs, according to the extent and site of bleeding. This early treatment is possible by home therapy. Life-threatening bleedings such as bleeding of the central nervous system, throat, or neck should be treated as emergency, immediately before evaluation. Recently, the World Federation of Hemophilia published a guideline for the management of hemophilia, which also suggested a good standard for the care of bleeding patients. In this article, the author introduce coagulation factors available in Korea for the treatment of hemophilia patients, including bypassing agents for inhibitor patients, and discuss practical points for the treatment of hemophilia patients in Korea.