ABSTRACT
Introduction: Acquired hemophilia A (AHA) is a rare hemorrhagic disease in nonhemophiliacs (1 case/106 persons per year) due to autoantibodies/inhibitors against factor VIII. The disease occurs more often in the elderly, it is usually idiopathic and it causes sudden skin and mucosal bleeding. The aim of this study was to describe a case of AHA, diagnosed during hospitalization for COVID-19. Method(s): A female patient, 87 years old was hospitalized for COVID-19. She had no personal or family history of spontaneous bleeding and was not under anticoagulant therapy. Routine coagulation parameters, mixing test-aPTT and activity of coagulation factors were performed in ACL-TOP 750 (IL). Result(s): The first bleeding episode happened two months ago when the patient underwent coronary angiography and started bleeding mainly at the catheterization point (left radial artery). Three hospitalizations followed due to extensive ecchymoses and recurrent bleeding episodes at multiple sites of venipuncture, treated with hemostatic suturing and transfusions. The patient was subsequently admitted to our hospital due to COVID-19 infection and anemia. She presented with an isolated prolongation of activated partial thromboplastin time (aPTT) (>60) since the day of her first admission. In our hospital, a mixing-test of aPTT was performed: aPTTmix-direct = 40 [Rosner-Index = 11.45% (< 12% = correction)] aPTTmix-after two hours of incubation at 37degreeC = 62.5 [(Rosner-Index = 36.45% (>15% = no correction)] Therefore, the probable presence of a coagulation factor inhibitor was indicated. No lupus anticoagulant was detected (DRVVT-TR = 0.9). A reduced activity of FVIII = 1.9% (m.p.= 50-150%) was found and she was immediately treated with prednisolone, with positive clinical response, cessation of bleeding episodes and normalization of Hct and aPTT. Conclusion(s): AHA is the most common type of acquired hemophilia. An isolated prolongation of aPTT is usually due to anticoagulants mainly heparin. If not, it may indicate a clotting-factor deficiency or the presence of an inhibitor, that is either specific (e.g. antibody to factor VIII/AHA) or nonspecific (e.g. lupus anticoagulant). The mixing-test aPTT should be routinely used in these cases to detect AHA, a rare disease usually underdiagnosed that can be fatal without early recognition and appropriate management.