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A 71-year-old man was diagnosed with T-Large granular lymphocytic (LGL) leukemia, which usually represents a relatively indolent clinical course. While the clinical manifestation of this patient we report herein was aggressive with lasting fever, splenomegaly and hemophagocytic lymphohistiocytosis (HLH). T-cell immunophenotype was CD3+CD4-CD8-CD5-CD7-TCRαβ+. After comprehensive evaluation, an adjusted chemotherapy regimen CEOP (cyclophosphamide, vincristine, etoposide, prednisone) with etoposide, a potential effective regimen for HLH was administrated to the patient. Although he received intensive regimen, the patient showed drug resistance and disease progression with central nervous system (CNS) involvement during treatment and showed only transiently response to intrathecal methotrexate, cytarabine and dexamethasone. Therefore, considering the refractory elderly patient with fragile physical condition, metronomic regimen T-PEPC (oral administration of thalidomide, prednisone, cyclophosphamide, etoposide and methyhydrazine) was recommended, which refers to the frequent even daily administration of cytotoxic drugs at comparatively low doses with minimal or prolonged drug-free breaks. The patient responded well to this treatment and remained symptom-free for 8-month follow-up. To our knowledge, this is the first case of reporting this unique immunophenotype of dual CD4-/CD8- with aggressive clinical course and CNS involvement that successfully treated with metronomic regimen, suggesting that low dose metronomic regimen could be a better option for elderly patient with aggressive T-LGL leukemia.

oncotarget

Metronomic regimen as an effective treatment for aggressive T-LGL leukemia with central nervous system infiltration: clinical experience and review of literature