Acquired hemophilia A as a cause of recurrent bleeding into the pleural cavity – and literature review

Abstract

Acquired hemophilia A is a coagulation disorder caused by autoantibodies against blood coagulation factor VIII. The first sign of this disease is often massive bleeding, which can affect patients after routine procedures. The parameter which indicates the presence of this condition is isolated prolonged activated partial thromboplastin time (APTT). The present article describes a case of a 32-year-old man with acute interstitial pneumonia and pleural effusion, in whom a massive hemothorax appeared after thoracocentesis; active bleeding was observed after the introduction of a chest tube. The patient was operated on, and no pinpoint bleeding was discovered during the procedure. Active bleeding was still taking place postoperatively. The patient underwent another operation after 6 days. Once more, no pinpoint bleeding was found. Prolonged APTT was observed. The activity of blood coagulation factor VIII was 3.04%. The presence of antibodies against factor VIII was confirmed, and acquired hemophilia was diagnosed. The article also includes an analysis of the literature on acquired hemophilia.

Hemorrhagic pericardial effusion as the debut of acquired hemophilia in a chronic lymphocytic leukemia patient

“…[ 2 , 5 , 14 ] In the course of this disease, bleeding into the thoracic cavity is very uncommon (I approximately 1% of cases); 3 cases of hemorrhagic pleural effusion or hemothorax have been reported in the literature. [ 9 ] Potentially fatal bleeding into the thoracic cavity caused by AHA may appear after surgical procedures performed on the chest. [ 15 ] Previously, in 1 patient diagnosed with myeloma multiple, hemarthrosis and hemorrhagic pericardial effusion were in association with AHA [ 16 ] (Table 1 ).…”

“…[ 3 ] Bleeding into the thoracic cavity (e.g., hemorrhagic pleural effusion or pericardial effusion) and intracranial hemorrhage may be fatal, but are very uncommon, occurring in only approximately 1% of cases. [ 9 ] AHA should be suspected in patients with a recent onset of abnormal bleeding, an isolated prolongation of activated partial thromboplastin time (aPTT) and normal prothrombin time (PT). [ 10 ] Laboratory tests indicating AHA include a mixing study consistent with an inhibitor, a negative result for lupus anticoagulant (LA), and low levels of FVIII.…”