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Case Study: Rare Richter Transformation of CLL Involving the Ear

By: Celeste L. Dixon
Posted: Monday, May 3, 2021

The description of a patient who experienced Richter transformation of chronic lymphocytic leukemia (CLL) to aggressive non-Hodgkin lymphoma (NHL), initially misdiagnosed as acute otitis externa, provides a lesson to other clinicians who might encounter such a case. Georgia Kaiafa, MD, PhD, of Aristotle University in Thessaloniki, Greece, and colleagues illustrated and discussed the encounter with the 75-year-old female patient in the Polish Archives of Internal Medicine.

“Prompt recognition may be key to prolong survival,” stated Dr. Kaiafa and colleagues. “The most common clinical symptoms and signs include severe otalgia, otorrhea with exudative discharge, ear canal erythema and edema, sensorineural hearing loss, difficulty with mastication, and regional lymphadenopathy.” Although management varies by NHL subtype and other factors, the primary therapeutic approach is immunopolychemotherapy, they added.

The diagnosis of acute otitis externa came after an ear, nose, and throat consultation for ear pain and hearing loss. However, 3 years earlier, the patient had been diagnosed with CLL (Rai stage 1, Binet stage A). One month earlier, after a marked increase in her lymphocyte count, the patient had begun chemoimmunotherapy with fludarabine, cyclophosphamide, and rituximab.

The ear anomalies signaled NHL localization in the external auditory canal, a rare occurrence often misdiagnosed as acute otitis externa. The combined systemic antimicrobial and topical treatment were not effective. The patient was hospitalized and developed marked regional lymphadenopathy. The hospital workup identified no pathogen, so she received empiric antimicrobial treatment with meropenem and clindamycin.

Ultimately, noted the team, “an external auditory canal biopsy revealed diffuse infiltration of the cutaneous lamina propria by neoplastic lymphoid cells, with histologic and immunohistochemical characteristics compatible with high grade NHL.” The patient died of sepsis 3 weeks after hospitalization.

Disclosure: The study authors reported no conflicts of interest.



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