Acquired Hemophilia A Newly Diagnosed in an Elderly Patient − A Case Report
At the XXVIIIth Pařízek Days held in Ostrava, a case of acquired hemophilia A, recently addressed at this institution, was presented in the afternoon session on March 17, 2022, by Assoc. Prof. Petr Dulíček, MD, PhD., from the 4th Department of Internal Medicine, Charles University Faculty of Medicine and University Hospital in Hradec Králové.
Medical History
The patient was an 87-year-old woman referred from a regional hospital due to bleeding symptoms. The family history was insignificant in terms of bleeding symptoms, but her personal medical history included rheumatoid arthritis treated for the last 7 months, as well as Sjögren's syndrome and other chronic conditions. Her pharmacological history included Plaquenil, Prednisone, Cotrimoxazole, Zulbex, and Egilok. Her allergy history included an allergy to iodine. According to the information, the woman had not contracted COVID-19 so far. Until this time, she had been leading a very active life.
Clinical Picture and Laboratory Diagnostics
Bleeding symptoms appeared in November 2021, about 14 days before admission to the hospital, when she observed recurrent bleeding from hemorrhoids. A week later, hematuria with abdominal pain occurred, for which she received intramuscular analgesics once. Subsequently, extensive bleeding skin manifestations appeared at the injection site and elsewhere on the body.
This led to primary examination at the regional hospital, where isolated prolongation of aPTT (96.9 s) and anemia with hemoglobin of 79 g/l were demonstrated. Targeted coagulation examination showed the level of coagulation factor VIII (FVIII) at 1.1% and the presence of FVIII inhibitor at a titer of 16.2 BU.
Treatment Course
The diagnosis of acquired hemophilia A (AHA) was established, and the patient was treated at a specialized center with continuous infusion of recombinant FVIIa (initially at a dose of 8 µg/kg/hr) for hemostasis, and eradication therapy of the inhibitor was initiated with a combination of intravenous corticosteroids and oral cyclophosphamide (2 × 50 mg). Due to bleeding manifestations, the dose of the bypassing agent had to be increased by about half after 6 days.
Complications
About 14 days after admission, PCR positivity for SARS-CoV-2 was demonstrated, and the patient was treated with remdesivir. During the course of treatment, she also developed ESBL-positive Klebsiella sepsis with a good response to antibiotics. The eradication of the inhibitor was successful, and in February 2022, the woman was in remission of the disease (FVIII level 81%) and was discharged to home care. However, she stayed there only for a few days because she fell and suffered a subcapital fracture of the femoral neck. At that time, the AHA remission persisted, so she was operated on acutely and without complications. In the early postoperative period, however, cardiopulmonary arrest occurred, the etiology of which was not entirely clear. The patient was successfully resuscitated, but there was no significant recovery during subsequent care, and the woman is now bedridden with a tracheostomy.
Conclusion
This is a typical case of acquired hemophilia A in older age, which was successfully treated. Unfortunately, the subsequent post-traumatic complication has left the woman bedridden and significantly worsened her prognosis.
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Source: Dulíček P. Acquired Hemophilia: Case Report. XXVIIIth Pařízek Days, March 17, 2022.
Source of illustrative image: Dulíček P. Acquired Hemophilia: Case Report. XXVIIIth Pařízek Days, March 17, 2022. Published with permission.
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