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

Abstract

Background: 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.

Clinical findings: 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 (<20% of cases).

Conclusion: A multidisciplinary team is needed to diagnose and manage AHA effectively. The use of anticoagulants may lead to the misdiagnosis of clinical symptoms. Chronic lymphocytic leukemia is one of the main causes of hematological malignancies associated. The specific treatment of CLL is still recommended in the event of active disease.

Figure 1

Chest radiograph. Frontal (A) and lateral (B) chest radiographs showed an enlarged cardiac silhouette with an increase in the transverse diameter but no increase in its height, creating a globular morphology (“water bottle” sign). This sign is present when there is a large pericardial effusion.

Figure 2

Transthoracic echocardiography in apical 4-chamber view showing pericardial effusion. The echocardiograph showed a large circumferential pericardial effusion (>25 mm at end-diastole).

Figure 3

Spontaneous sublingual hematoma.

Figure 4

Changes in FVIII activity and inhibitor levels related to hemostatic and immunosuppressive therapy. Patient presented hemorrhagic pericardial effusion and a spontaneous sublingual hematoma. Inhibitor (7 BU) was detected and acquired hemophilia A was confirmed. Hemostatic treatment with rFVIIa (90 μ/kg/4 h) and corticosteroids (1 mg/kg) and cyclophosphamide (50 mg/day) (IST) was started. Due to favorable evolution, rFVIIa was reduced and stopped on day 10. Until the inhibitor had been eliminated by immunosuppressive therapy therapy, factor VIII activity levels did not increase. On day 14, corticosteroids were tapered. CC and immunosuppressive therapy were stopped on day 60 and complete remission was maintained. AAS was restarted on day 30 when hemostasis was safe and the inhibitor had been eradicated. rFVIIa = recombinant activated factor VII.

Figure 5

Acute deep vein thrombosis revealed by gray-scale ultrasound. Longitudinal (A) and transverse (B) gray-scale ultrasound images of the gemellar vein demonstrated an enlarged noncompressible vein with intraluminal echoes.