Acquired hemophilia A presenting as progressive intra-abdominal hemorrhage, muscle hemorrhage and hemothorax postpartum: A case report and literature review

Abstract

Acquired hemophilia A (AHA) is a rare antibody-mediated condition in which autoantibodies form against a coagulation factor, most commonly factor VIII (FVIII), causing severe coagulopathy. Here the present report presents a case of AHA in a 35-year-old postpartum woman with continuous polyserous bloody effusions who was admitted to the First Affiliated Hospital of Zhejiang Chinese Medical University (Hangzhou, China) in October 2017 without a history of trauma, anticoagulation treatment or coagulopathy. At presentation, the patient's hemoglobin level was low (70 g/l; normal range: 115-150 g/l) g/l, blood pressure was 89/58 mmHg (normal range, 90-140/60-90 mmHg), and activated partial thromboplastin time was 68.4 sec (normal range: 25.0-36.0 sec), with a normal international normalized ratio (0.94; normal range, 0.8-1.2). The reaction time in thrombography was prolonged (35.8 min; normal range: 5-10 min), coagulation FVIII had markedly decreased activity (12.6%; normal range, 60-150%), and FVIII inhibitor had a high titer [7.4 Bethesda units (BU)/ml; normal range, 0-0.6 BU/ml]. Notably, the patient's autoantibody level was markedly higher than normal (1:320; normal range: <1:100). The patient was successfully treated with bleeding control, eradication of FVIII inhibitor, and treatment of the underlying disease. To the best of our knowledge, this is the first case of AHA with polyserous bloody effusions in a patient with an autoimmune disorder during the postpartum period. Reports of such rare cases will aid the characterization of disease pathogenesis, which may in turn lead to the recognition and timely treatment of this rare disorder.

Keywords: China; acquired hemophilia A; hemorrhage; polyserous effusions; premature delivery.

Figure 1.

Contrast-enhancement computed tomography scan showing (A and B) pleural effusion, (C) right abdominal cavity effusion and (D) pelvic effusion and exudative lesions of the soft tissues surrounding the right groin and thigh.

Figure 2.

(A) Color doppler ultrasonography showing mild mitral valve reflux. (B) Tissue doppler imaging showing tricuspid valve regurgitation, high pulmonary artery systolic blood pressure and reduced diastolic left ventricular function. The parameters of cardiac function were as follows: Right ventricular internal dimension in diastole, 26.1 mm; intraventricular septum in diastole, 7.6 mm; left ventricular internal dimension-in diastole, 43.1 mm; left ventricular posterior wall dimension, 7.6 mm; left ventricular internal dimension in systole, 23.4 mm; heart rate, 84 bpm; aorta, 23.1 mm; left atrium, 32.5 mm; fractional shortening, 45.7%; ejection fraction, 77.4%).

Figure 3.

Thromboelastography showing a prolonged R-time (35.8 min) and K-time (15.3 min), a narrowed angle (35.2°), decreased MA (44.5 mm), decreased CI (−28.8) and normal clot lysis, which is reported in terms of EPL (0.0%) and LY30% (0.0%). R, reaction time; K, coagulation time; deg, degree; MA, maximum amplitude; CI, coagulation index; EPL, estimated percent lysis; LY30%, percent clot lysis at 30 min after maximum amplitude; A, amplitude.

Figure 4.

Schematic flowchart for the diagnosis of acquired hemophilia, lupus anticoagulant and single factor deficiency. FVIII, factor VIII; aPTT, activated partial thromboplastin time.