Large granular lymphocyte leukemia: A study of nine cases in a Chinese population

Abstract

Large granular lymphocyte (LGL) leukemia is a neoplastic disorder of lymphocytes that is characterized by the presence of prominent cytoplasmic granules, and involves the proliferation of at least two distinct cell types, T cells and natural killer (NK) cells. The authors report the clinical and pathologic features of 9 Chinese patients with LGL leukemia, who represented 14% of 64 cases of chronic lymphoproliferative disorders diagnosed at their centers in 3 years. Three different groups could be defined on immunophenotypic and clinical grounds. The first group of 4 cases were CD2+CD3+CD4-CD8+. With the exception of a pediatric case, these cases ran an indolent course that was similar to the T-cell LGL leukemia most common in Western patients. However, thrombocytopenia and pure red cell aplasia were more common in the patients in this study, which was similar to the experience in Japanese patients. The second group of two cases were CD2+CD3+CD4+CD8-, and appeared to have worse outcomes than the first group. The third group of 3 cases were CD2+CD3-CD4-CD8-CD56+. Although phenotypically similar to the NK-cell LGL leukemia reported in Western patients, these cases were clinically more aggressive than their Western counterparts. This study is the first to report comprehensively the different types of LGL leukemias in Chinese patients, and provides useful information on the similarities and differences of these disorders as compared to those cases in the West.