Case Presentation: A 68-year-old Spanish speaking female with a history of chronic lymphocytic leukemia (CLL), in remission, and autoimmune hemolytic anemia (AIHA) presented to the ED with left sided neck pain, edema, and hoarseness. These symptoms had been present for the past four months despite three courses of antibiotics. Steroids given in the ED improved these symptoms, and the patient was admitted for an incidental finding of hyponatremia. Physical exam was significant for tender cervical lymphadenopathy and a hoarse voice. Imaging confirmed diffuse lymphadenopathy with an incidental finding of sinusitis, for which the patient was started on antibiotics. Chest x-ray and respiratory viral testing were unremarkable. Hematology was consulted for labs concerning for recurrence of CLL and AIHA. On the second day of hospitalization, patient endorsed a sore throat and dyspnea. She was speaking in full sentences with no increased work of breathing and an unremarkable lung exam. One hour later a rapid response was called with concern for impending airway compromise. A direct laryngoscopy at bedside showed significant supraglottic edema. She was transferred to the ICU and steroids were initiated with symptom improvement. This was thought to be due to an allergic reaction. Four days later, about 24 hours after stopping steroids, the patient again endorsed a subjective sense of airway swelling with tripoding; laryngoscopy confirmed worsening supraglottic edema. Steroids, racemic epinephrine, and heliox were initiated. Allergy was consulted and thought that her low C4 was consistent with acquired C1 esterase inhibitor deficiency related to a likely recurrence of CLL. Berinert was initiated with significant improvement in symptoms. She was discharged on steroids, Rituximab, plan for Icatibant as rescue medication, and hematology and allergy follow up.
Discussion: This case highlights a rare cause of angioedema known as acquired C1-inhibitor deficiency (ACID). This can be acquired in the setting of lymphoproliferative disorders. The edema is self-limited, but can be fatal with attacks of the upper airway causing asphyxiation. Diagnosis was delayed due to the rarity of ACID, subjective presentation, and language barriers. The patient could only explain the swelling in her throat as a sore throat, difficulty swallowing, and pain. These symptoms are vague and subjective, likely leading to multiple prior healthcare visits believing the etiology to be infectious and treating with repeated antibiotic courses. It was not until the patient was visually in distress that direct visualization revealing supraglottic edema was performed. In this case, using a translator was necessary for communication with the patient, but there is a risk that important information may not be translated accurately due to limitations in languages. This case shows the importance of clarifying a patient’s symptoms and improving communication when using an interpreter such as: using simple terms, straightforward language, not interrupting, and talking slowly.
Conclusions: ACID is a rare etiology of angioedema and can be fatal. It can be difficult to diagnose ACID and other diseases that present with mainly subjective symptoms, especially when a language barrier is present. When using a translator, information can be lost or miscommunicated. Providers should try to utilize the skills represented above to improve communication when a translator is needed.