SICKLE β-thalassemia, a hemoglobinopathy closely related to sickle cell disease, demonstrates vaso-occlusive phenomena similar to homozygous sickle cell anemia. We describe a patient who experienced severe laryngeal edema secondary to sludging of sickled RBCs (drepanocytes) in the capillaries and lymphatic channels in the laryngeal microvasculature. To our knowledge, this finding has not been previously reported in the literature.
Report of a Case
A 29-year-old man with known sickle β-thalassemia disease came to the emergency room with acute dyspnea on June 26, 1980, and was admitted to the hospital. Indirect laryngoscopy showed marked edema of the left arytenoid mucosa and false vocal cord. The true vocal cord was obscured by a ventricular band. The glottic opening was diminished on deep inspiration. The right side of the larynx, however, appeared normal. A regimen of 100 mg of hydrocortisone every six hours produced some abatement of the patient's symptoms. Two days after admission,
Greene GS, DellaVecchia M, Shea FJ. Laryngeal Edema Associated With Sickle β-Thalassemia Disease. JAMA. 1982;247(9):1318. doi:10.1001/jama.1982.03320340072042