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Ear involvement in acute promyelocytic leukemia at relapse: a disease-associated 'sanctuary'?
M Breccia1, M C Petti, A M Testi
1Department of Cellular Biotechnologies, University La Sapienza, Rome, Italy.
Abstract:
Extramedullary (EM) involvement occurs infrequently in acute promyelocytic leukemia (APL) and usually involves skin and CNS. We describe seven patients (four observed at a single institution) who relapsed in various sites of the auditory apparatus, including the external canal and middle ear (temporal bone). Front-line treatment included ATRA and chemotherapy (six patients) or chemotherapy alone (one patient). Three patients had concomitant hematologic relapse, two had molecular relapse and two were in hematologic and molecular remission when ear localization was documented. Local symptoms that stimulated further diagnostic studies included ear bleeding/discharge in the first patient, but were mild in the others (hypoacusia, five patients; earache, two patients). Ear involvement by leukemia was documented by histological and/or molecular studies after local surgery in five cases, and by CT scan or NMR in the remaining patients. We suggest that the ear might represent a specific sanctuary for disease involvement in APL.
Insights
Extramedullary involvement in acute promyelocytic leukemia (APL) is rare. This study highlights seven patients with APL relapsing in the auditory apparatus, suggesting the ear may be a sanctuary site for leukemia.
Area of Science:
- Hematology
- Oncology
- Otolaryngology
Background:
- Extramedullary (EM) involvement in acute promyelocytic leukemia (APL) is uncommon, typically affecting the skin and central nervous system (CNS).
- Understanding atypical sites of relapse is crucial for comprehensive patient management.
Observation:
- Seven patients with APL presented with relapse in the auditory apparatus, including the external ear canal and middle ear (temporal bone).
- Symptoms included ear bleeding/discharge, hypoacusis, and earache, with some patients in hematologic or molecular remission at diagnosis of ear localization.
- Diagnosis was confirmed via histological, molecular, CT, or NMR studies.
Findings:
- The auditory apparatus, specifically the ear, can serve as a sanctuary site for relapse in acute promyelocytic leukemia.
- This EM involvement can occur even in the absence of overt hematologic relapse.
Implications:
- Clinicians should consider the auditory system when evaluating APL patients for extramedullary relapse.
- Further investigation into the ear as a sanctuary site may inform novel diagnostic and therapeutic strategies for APL.