A Multifactorial Case of Acquired Hemophilia A

TL;DR

A 73-year-old woman with multiple health issues developed a rare autoimmune bleeding disorder, which led to severe complications and ultimately death despite treatment.

Contribution

This case study presents a complex clinical scenario of acquired hemophilia A with comorbidities, emphasizing management challenges and outcomes.

Findings

The patient's AIHA was complicated by heart disease, diabetes, hypertension, and a positive lupus anticoagulant test.

Despite treatment with corticosteroids, cyclophosphamide, rituximab, and factor VIIA, she had recurring bleeding and complications.

The case underscores the need for early diagnosis, multidisciplinary care, and individualized treatment in AIHA.

Abstract

Acquired hemophilia A (AIHA) is a rare autoimmune bleeding disorder in which the body develops autoantibodies against factor VIII, leading to spontaneous bleeding. It has an incidence of about one person per million each year. AIHA carries high risks; these symptoms are only exacerbated with other comorbidities, as explored in this patient, a 73-year-old female patient with a history of heart disease, diabetes, hypertension, and a positive lupus anticoagulant (LA) test. She presented with a rapidly growing neck hematoma that compromised her airway, requiring immediate intubation and admittance to the ICU. Laboratory tests showed a severely prolonged activated partial thromboplastin time (aPTT) and critically low factor VIII levels, confirming the diagnosis of AIHA. Despite early treatment with corticosteroids, cyclophosphamide, rituximab, and recombinant factor VIIA, she experienced repeated bleeding episodes and developed complications such as neutropenic fever and refractory depression. Ultimately, due to the cumulative impact of these complications, she was transitioned to palliative care and died. This case highlights the importance of prompt diagnosis and treatment, the difficulties of balancing immunosuppression and bleeding risk, and the impact significant illnesses can have on mental health. Early recognition, multidisciplinary management, and individualized treatment strategies are crucial to improving outcomes in patients with this rare disorder.

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