A Presumed Sickle Cell Anemia Crisis Revealed To Be Medium Vessel Vasculitis

Abstract

A 24-year-old male sickle cell anemia patient presented with acute abdominal pain. Computed tomography (CT) demonstrated signs of bowel ischemia about the terminal ilium. He underwent bowel resection and anastomosis. Pathology of the resected bowel showed acute inflammation at the site of bowel perforation. This was thought to be secondary to bowel infarction from sickle cell vasculopathy. Despite the surgical intervention, the patient’s symptoms continued to worsen. He also developed bilateral toe pain during the same hospital stay. Reviewing the patient’s CT lower extremity runoff revealed no vascular thrombosis but rather medium vessel changes. The intra-abdominal arterial branches and the lower extremity vessels showed intermittent areas of vascular narrowing, wall thickening, and associated micro-aneurysms mainly of the distal hepatic arterial branches. These vascular changes posed a diagnostic dilemma as it is inconsistent with sickle cell anemia, which is known to cause vascular angiopathy as the underlying etiology of vaso-occlusive crisis. The literature lacked reports of any specific intra-abdominal vascular findings by imaging in sickle cell anemia. With the continued worsening of the patient’s condition, vasculitis was considered as an alternative diagnosis. The patient was empirically treated with steroids after which his symptoms improved. Unfortunately, he passed away after developing a large intracranial hematoma days after the initiation of steroid therapy. This report highlights the diagnostic dilemma of vaso-occlusive crisis versus vasculitis in sickle cell anemia patients.