CO-86 | A RARE CASE OF ACQUIRED HEMOPHILIA A ASSOCIATED WITH MYELOPROLIFERATIVE NEOPLASM

A. Ierardi, A. Strangio, M. Leotta, R.C. Santoro | Hemophilia, Hemostasis and Thrombosis Unit, Azienda Ospedaliero Universitaria Renato Dulbecco, Catanzaro, Italy

Introduction. Acquired hemophilia is a rare bleeding disorder characterized by the development of inhibitors against factor VIII, with idiopathic cases accounting for approximately half of all occurrences. The association between acquired hemophilia and chronic myelomonocytic leukemia (CMML) is less common. CMML is an aggressive clonal stem cell disorder categorized among myelodysplastic/myeloproliferative overlap neoplasms with distinct molecular and clinical profiles and is often associated with various hematological abnormalities.
Case Report. We present the case of an 85-year-old female patient with a complex medical history, including chronic respiratory failure, hypertension, and cerebral cavernomas, accompanied by epilepsy and essential tremor. The patient was admitted for anemia and widespread hematomas, during which she was diagnosed with acquired hemophilia. A computed tomography (CT) scan of the brain revealed an ischemic focus in the pericapsular area. The day prior to the diagnosis, the patient experienced a cardiac arrest that required defibrillation. At presentation, factor VIII levels were at 3.8%, and the inhibitor level was 2.4 Bethesda Units (BU). Treatment included porcine recombinant factor VIII at a dosage of 100 mg/kg for a total of 10 administrations, along with immunosuppressive therapy using steroids (prednisone 1 mg/kg/day). This treatment led to a rapid resolution of both the hematomas and the inhibitor, with the latter disappearing after 30 days of immunosuppressive therapy. Recombinant factor VIIa (rFVIIa), activated prothrombin complex concentrate (aPCC), or recombinant porcine factor VIII (rpFVIII) are generally used for managing bleeding in AHA patients. In this case, we opted for rpFVIII due to the patient's high thrombotic risk.
Discussion. The mechanism behind secondary acquired hemophilia A (AHA) due to the presence of chronic myelomonocytic leukemia (CMML) or myelodysplastic syndromes (MDS) remains unclear. In cases of hematological malignancies complicated by AHA, lymphoid malignancies—including lymphoma, chronic lymphocytic leukemia, and macroglobulinemia—are more commonly observed than myeloid malignancies. However, it is still possible for myeloid malignancies to infrequently complicate with AHA. This could be due to elevated levels of inflammatory cytokines such as interleukin-6 (IL-6) or tumor necrosis factor-alpha, which may activate autoimmune processes. Nevertheless, the precise mechanism responsible for this phenomenon is not well understood.