Abstract
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 methylhydrazine) 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.
Highlights
Large granular lymphocytic(LGL) leukemia is a rare T or NK clonal lymphoproliferative disorder, whose clinical presentation is dominated by a variety of autoimmune disorders including pure red cell aplasia, Sjogrens Syndrome and rheumatoid arthritis (RA) [1]
computed tomography (CT) scan of brain indicated no spaceoccupying lesions, but cerebrospinal fluid test showed that cell count was 540 /μl, Pandy test was positive, protein in cerebrospinal fluid was 0.95g/L, hypercellular fluid was infiltrated with mononuclear cells which presented with abundant cytoplasm and azurophilic granules (Figure 3), with its immunophenotype in accordance with bone marrow, the diagnosis of T-cell LGL (T-LGL) leukemia with central nervous system (CNS) involvement was confirmed
The patient we reported, with typical morphological feature, unique immunophenotyping by flow cytometry and T cell clonality demonstrated by TCR rearrangement plus clinical presentation, could make a definite diagnosis of T-LGL leukemia
Summary
Large granular lymphocytic(LGL) leukemia is a rare T or NK clonal lymphoproliferative disorder, whose clinical presentation is dominated by a variety of autoimmune disorders including pure red cell aplasia, Sjogrens Syndrome and rheumatoid arthritis (RA) [1]. CT scan of brain indicated no spaceoccupying lesions , but cerebrospinal fluid test showed that cell count was 540 /μl, Pandy test was positive, protein in cerebrospinal fluid was 0.95g/L, hypercellular fluid was infiltrated with mononuclear cells which presented with abundant cytoplasm and azurophilic granules (Figure 3), with its immunophenotype in accordance with bone marrow, the diagnosis of T-LGL leukemia with CNS involvement was confirmed. Given that the disease progressed with CNS involvement during chemotherapy and the patient with fragile physical condition, intensive intravenous chemotherapy was not appropriate Into this embarrassing situation, oral metronomic regimen T-PEPC (oral administration of prednisone 40mg, cyclophosphamide 50mg, etoposide 50mg, methyhydrazine 50mg, thalidomide 100mg every day) targeted both malignant cells and microenvironment was considered to control progressive disease with low toxicities.
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