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Central Nervous System Progression in Folliculotropic Mycosis Fungoides
Cher Ying Foo1, Aditya Sanjeevi1, Harkarandeep Singh1
1Department of Internal Medicine, Rochester General Hospital, Rochester, New York, USA, rochesterregional.org.
Central nervous system involvement is rare in folliculotropic mycosis fungoides (FMF), a type of cutaneous T-cell lymphoma (CTCL). Intrathecal chemotherapy effectively treated CNS progression in a patient with FMF.
Area of Science:
- Neurology
- Oncology
- Dermatology
Background:
- Central nervous system (CNS) involvement in cutaneous T-cell lymphomas (CTCL) is rare and linked to poor prognosis.
- Folliculotropic mycosis fungoides (FMF), a rare CTCL subtype, can exhibit aggressive behavior.
- Risk factors for CNS progression in primary CTCL remain poorly understood.
Purpose of the Study:
- To report a rare case of CNS progression in a patient with FMF.
- To illustrate the diagnostic findings and management of CNS involvement in FMF.
- To highlight the efficacy of intrathecal chemotherapy in managing CNS FMF.
Main Methods:
- Case report of a 75-year-old male with Stage IA FMF.
- Diagnostic workup included CSF analysis, flow cytometry, and brain MRI.
- Treatment involved high-dose steroids and intrathecal chemotherapy (methotrexate and cytarabine).
Main Results:
- Patient presented with progressive neurologic decline and eosinophilia.
- CSF analysis revealed a monoclonal T-cell population consistent with FMF.
- Brain MRI showed CNS lesions indicative of FMF progression.
- Intrathecal chemotherapy led to significant neurologic improvement and resolution of atypical T-cells.
Conclusions:
- CNS progression of FMF can occur even with controlled skin disease and no lymphatic involvement.
- Intrathecal chemotherapy is an effective treatment for CNS complications of FMF.
- This case emphasizes the importance of considering CNS involvement in FMF patients with neurologic symptoms.
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