Acquired hemophilia A in solid cancer: Two case reports and review of the literature

Abstract

Acquired hemophilia A (AHA) is a rare, hemorrhagic autoimmune disease, whose pathogenesis involves reduced coagulation factor VIII (FVIII) activity related to the appearance of inhibitors against FVIII. Common etiological factors include autoimmune diseases, malignancy, and pregnancy. We report two cases of AHA in solid cancer. The first case is a 63-year-old man who developed peritoneal and intestinal bleeding after gastrectomy for gastric cancer. He was diagnosed with AHA, and was treated with prednisone, followed by cyclophosphamide. In the second case, a 68-year-old man developed a subcutaneous hemorrhage. He was diagnosed with AHA in hepatocellular carcinoma on CT imaging, and treated with rituximab alone. Hemostasis was achieved for both patients without bypassing agents as the amount of inhibitors was reduced and eradicated. However, both patients died within 1 year due to cancer progression. Successful treatment for AHA in solid cancer can be difficult because treatment of the underlying malignancy is also required.

A Unique Presentation of Spontaneous Compartment Syndrome due to Acquired Hemophilia A and Associated Malignancy: Case Report and Literature Review

“…Malignancy remains a significant cause of AHA with an estimated 10-15% of patients with AHA having cancer as their underlying diagnosis [12]. AHA has been reported with solid and hematologic malignancies and has a worse prognosis with solid malignancies as they are typically detected at a later stage [9]. It is not classically associated with any one cancer, such as the association of Lambert-Eaton myasthenia syndrome with small-cell lung carcinoma, but is most commonly reported in association with lung and prostate cancer [9].…”

“…AHA has been reported with solid and hematologic malignancies and has a worse prognosis with solid malignancies as they are typically detected at a later stage [9]. It is not classically associated with any one cancer, such as the association of Lambert-Eaton myasthenia syndrome with small-cell lung carcinoma, but is most commonly reported in association with lung and prostate cancer [9]. Notably, there is an association with AHA secondary to malignancy and higher rates of recurrent and severe disease [8,9].…”

“…It is not classically associated with any one cancer, such as the association of Lambert-Eaton myasthenia syndrome with small-cell lung carcinoma, but is most commonly reported in association with lung and prostate cancer [9]. Notably, there is an association with AHA secondary to malignancy and higher rates of recurrent and severe disease [8,9]. In patients who are found to have treatment-resistant AHA, it is important for the clinician to investigate for underlying malignancy.…”

“…Additionally, in patients who have underlying solid malignancies, such as our patient, malignancies are usually detected at more advanced stages. These patients thus have a worse prognosis than those with hematologic malignancies [9]. It is currently not understood what causes the pathogenesis of AHA in patients with cancer, although it has been theorized to be due to immune system alteration in cancer patients or as a potential sequalae of chemotherapy [9].…”

“…A recent systematic literature review reports a large number of AHA patients with solid cancers, with the most common being prostate cancer (25.3%), lung cancer (15.8%), followed by colon cancer (9.5%) [8]. AHA in association with malignancy has been described in several case reports, including gastric, hepatocellular, thyroid, breast and lung carcinomas [9]. In this paper, we describe a rare case of probable colorectal carcinoma detected in a patient with AHA.…”