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Sensorineural Hearing Loss in Juvenile CML: A Rare Case Report in Surabaya, Indonesia
Ihda Kartika Syamsuddin1, Paulus Budiono Notopuro2
1Department of Clinical Pathology, Faculty of Medicine Universitas Airlangga, Surabaya, Indonesia.
Abstract:
Chronic myeloid leukemia (CML) is a type of myeloproliferative neoplasm (MPN) in which granulocytes are the primary proliferating cells. CML in pediatric patients (juvenile CML) is an uncommon MPN, representing 2-3% of children newly diagnosed with leukemia. Sensorineural hearing loss that progresses rapidly is an uncommon early sign of a hematological disorder in patients with CML. This article presents the case of a 10-year-old patient with complaints of bilateral hearing loss for 2 weeks. Pure-tone audiometry indicated sensorineural hearing loss of the left ear and mixed hearing loss of the right ear. After an in-depth evaluation of a bone marrow smear and positivity for the BCR-ABL fusion gene, it was concluded that the patient had chronic-phase juvenile CML. However, hearing loss recovery after hydroxyurea therapy could not be observed because the patient died after suffering an uncontrolled seizure on day 14 of hospitalization.
Insights
Rapidly progressing hearing loss can be an early sign of juvenile chronic myeloid leukemia (CML) in children. This case highlights the importance of investigating hematological disorders in pediatric patients presenting with sensorineural hearing loss.
Area of Science:
- Hematology
- Pediatric Oncology
- Otolaryngology
Background:
- Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm (MPN) characterized by granulocyte proliferation.
- Juvenile CML, diagnosed in pediatric patients, accounts for 2-3% of childhood leukemia cases.
- Rapidly progressing sensorineural hearing loss is an uncommon but significant early indicator of CML.
Observation:
- A 10-year-old patient presented with bilateral hearing loss over two weeks.
- Audiometry revealed sensorineural hearing loss in the left ear and mixed hearing loss in the right ear.
- Bone marrow smear analysis and BCR-ABL fusion gene positivity confirmed chronic-phase juvenile CML.
Findings:
- The patient was diagnosed with chronic-phase juvenile CML.
- Despite initiating hydroxyurea therapy, hearing loss did not improve.
- The patient experienced an uncontrolled seizure and passed away on day 14 of hospitalization.
Implications:
- This case underscores the critical association between sensorineural hearing loss and juvenile CML.
- Early diagnosis and prompt management of CML in pediatric patients are crucial.
- Further research is needed to understand the mechanisms and potential treatments for hearing loss in CML.
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