Hemorrhagic pericardial effusion as the debut of acquired hemophilia in a chronic lymphocytic leukemia patient: A case report, and a review of acquired hemophilia A-related hematological malignancies

• Published in: Medicine (Baltimore) 2017-11, Vol.96 (47), p.e8669-e8669
• Main Authors: Bastida, José María, Cano-Mozo, María Teresa, Lopez-Cadenas, Felix, Vallejo, Victor Eduardo, Merchán, Soraya, Santos-Montón, Cecilia, González-Calle, David, Carrillo, Javier, Martín, Ana Africa, Torres-Hernández, Jose Angel, González, Marcos, Martín-Herrero, Francisco, Pabón, Pedro, González-Porras, Jose Ramon
• Format: Article
• Language: English
• Subjects: Aged; Antibodies - blood; Blood Coagulation Tests - methods; Clinical Case Report; Coagulants - administration & dosage; Cyclophosphamide - administration & dosage; Echocardiography - methods; Factor VIIa - administration & dosage; Factor VIII - analysis; Factor VIII - immunology; Hemophilia A - blood; Hemophilia A - complications; Hemophilia A - etiology; Humans; Immunosuppressive Agents - administration & dosage; Leukemia, Lymphocytic, Chronic, B-Cell - complications; Leukemia, Lymphocytic, Chronic, B-Cell - diagnosis; Male; Pericardial Effusion - diagnosis; Pericardial Effusion - etiology; Pericardial Effusion - physiopathology; Pericardiectomy - methods; Prednisone - administration & dosage; Radiography, Thoracic - methods; Recombinant Proteins - administration & dosage; Treatment Outcome
• ISSN: 0025-7974; 1536-5964
• DOI: 10.1097/MD.0000000000008669

Acquired hemophilia A (AHA) is a rare bleeding disease caused by autoantibodies against factor VIII. Spontaneous bleeding symptoms usually affect the skin and muscle, while pericardial effusion is an extremely rare manifestation. In the elderly, anticoagulant treatment is frequent and bleeding symptoms are usually associated with this. We report a hemorrhagic pericardial effusion as the AHA debut in a patient with untreated chronic lymphocytic leukemia and anticoagulated with apixaban for atrial fibrillation and chronic arterial ischemia. The patient was treated with recombinant activated factor VII to control the active bleeding and corticosteroids and cyclophosphamide to eradicate the inhibitor. In addition, a briefly review of hematological malignancies associated to acquired hemophilia was performed. PARTICULARITIES:: a) anticoagulant treatment may confuse the suspicion of AHA and its diagnosis; b) hemorrhagic pericardial effusion is an extremely rare presentation; c) bypassing agents raise the risk of thromboembolism; d) hematological malignancies rarely cause AHA (