Pharyngolaryngeal Hematomas in Hemophilia A
An 18-year-old man with hemophilia A presented to the otolaryngology clinic with a 4-day history of throat discomfort, painful swallowing, and difficulty moving his tongue. He had moderately severe hemophilia A with a baseline factor VIII activity level of 2 to 5% (reference range, 70 to 150). He administered recombinant factor VIII as needed for bleeding episodes and had started doing so 2 days before this presentation for a right neck hematoma. He recalled no recent neck or throat trauma, coughing, or choking. On physical examination, a resolving right anterior neck ecchymosis was seen (Panel A). Laryngoscopy showed multiple hematomas on the right oropharyngeal wall (Panel B, asterisk), lingual epiglottic surface (Panel B, arrowheads), and aryepiglottic fold (Panel C, arrows). The factor VIII activity level was 9%. In patients with hemophilia, pharyngeal or laryngeal bleeding may manifest as sore throat, odynophagia, vocal changes, or tongue discomfort. Factor replacement therapy should be administered urgently and empirically if there are diagnostic delays to avoid the progression of bleeding to airway compromise. The patient continued to receive recombinant factor VIII, and at follow-up 10 days later, his symptoms and hematomas had resolved.
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