A Case of CD4+T-Cell Large Granular Lymphocytic Leukemia
A 59-yr-old man with a skin rash that had been present for 6 months was admitted to the hospital for an evaluation. He was diagnosed with hypertension and diabetes mellitus. PB examination revealed the following: white blood cell count, 45×109 cells/L (consisting of 34% neoplastic lymphoid cells, 10% segmented neutrophils, 47% lymphocytes, 6% monocytes, and 3% eosinophils); hemoglobin, 131 g/L; mean corpuscular volume, 90.9 fL; and 419×109 platelets/L. Neoplastic lymphoid cells displayed large granules (). PB neoplastic lymphoid cells were surface CD3+, cytoplasmic CD3+, CD4+, CD7+, CD8-, CD16-, CD19-, CD20-, and CD56- (). Other laboratory results included the following: serum antinuclear antibody titer, 1:640; glucose, 1.39 g/L; hemoglobin A1c, 7.7%; total protein/albumin, 7.1/3.9 g/dL; AST/ ALT, 12/12 IU/L; and total bilirubin, 5 g/L. Multiple small enlarged lymph nodes (<1 cm in diameter) in both the inguinal and axillary areas, and mild hepatosplenomegaly were noted on the abdominal and pelvic computed tomography (CT) scans. A cutaneous nodule (1.5 cm in diameter) was also observed in the left suboccipital area, but this seemed to be a reactive enlargement of the lymph nodes. BM study revealed hypercellular marrow that consisted of 2.4% neoplastic lymphoid cells. The neoplastic lymphoid cells exhibited a medium size, irregularly shaped nuclei, a moderate amount of cytoplasm, and large granules in the cytoplasm (). Immunohistochemical analysis of the BM biopsy showed CD3+, CD4+, TCR βF1+, granzyme B+, and TIA1+ (). TCR γ gene rearrangement by BIOMED-2 PCR assays (InVivoScribe, San Diego, CA, USA) was negative. Cytogenetic analysis indicated an abnormal karyotype: 46,XY,inv(3)(p21q27),t(12;17)(q24.1;q21), del(13)(q14q22)[2]/46,XY[28]. The patient was diagnosed with CD4+ T-LGL and received chemotherapy (10.0 mg methotrexate/week for 4 months). After the treatment, PB examination indicated the following values: white blood cell count, 24×109 cells/L with 25% neoplastic lymphoid cells; hemoglobin, 137 g/L; and 395×109 platelets/L. The patient tolerated the treatment well, and his skin lesions improved.