Clonal Diseases of Large Granular Lymphocytes

Analysis of immunoglobulin and T cell receptor genes. Part II: Possibilities and limitations in the diagnosis and management of lymphoproliferative diseases and related disorders.

Perforin gene expression in granular lymphocyte proliferative disorders

Immunophenotypic characterization of leukemic cells has become essential for the diagnosis of acute leukemias (AL) and chronic lymphoproliferative disorders (CLPD). Immunophenotyping allows to classify AL according to (i) lineage assignement of the leukemic clone based on the degree of specificity (or “score”) of expressed markers, (ii) the differentiation level of the clone and (iii) the presence of irrelevant markers. In addition, some rare AL subtypes may be identified, such as M0, M6 “variant” and M7 FAB types, as well as “biphenotypic” (hybrid) AL. Finally particular immunophenotypic profiles are of pronostic value or associated with specific cytogenetic abnormalities. In leukemic phase CLPD setting, some circulating lymphoid cell immunophenotypic profiles are strongly correlated with morphology as defined by the FAB and REAL classifications. In addition, some marker expressions are of pronostic value. However, proper choices of sample nature, monoclonal antibodies and immunophenotyping methods are essential to improve quality, reliability and reproducibility of the results and must be carefully controlled in and between laboratories.

Immunophenotypic characterization of acute leukemias and chronic lymphoproliferative disorders: practical recommendations and classifications

To characterize T-cell large granular leukemia in Asia, 22 Chinese patients from a single institute were reported, together with an analysis of 88 Asian and 272 Western patients identified from the literature. In our cohort, anemia due to pure red cell aplasia (PRCA) occurred in 15/22 (68%) of cases, being the most common indication for treatment. Neutropenia was only found in 8/22 (36%) cases, and recurrent infections, the most important clinical problem in Western patients, were not observed. None of our cases presented with rheumatoid arthritis. These clinical features were consistently observed when compared with the 88 other Asian patients. Combined data from our cohort and other Asian cases showed that Asian patients, compared with Western patients, had more frequent anemia (66/110, 60% versus 113/240, 47%; p = 0.044), attributable to a much higher incidence of PRCA (52/110, 47% versus 6/143, 4%; p < 0.001). However, Western patients presented more frequently than Asian patients with neutropenia (146/235, 62% versus 33/110, 30%; p < 0.001) and splenomegaly (99/246, 40% versus 16/110, 15%; p < 0.001). Notably, Western patients were about eight to ten times more likely than Asian patients to have rheumatoid arthritis (73/272, 27% versus 4/106, 4%; p < 0.001) and recurrent infections (81/272, 30% versus 3/107, 3%; p < 0.001). These clinicopathologic differences have important implications on disease pathogenesis and treatment.

/pdf/t-cell-large-granular-lymphocyte-leukemia-an-asian-15hk0e982a.pdf

T-cell large granular lymphocyte leukemia: an Asian perspective

Ti (WT31)-negative, CD3-positive, large granular lymphocyte leukemia with nonspecific cytotoxicity.

nonspecific cytotoxicity Ti (WT31)-negative, CD3-positive, large granular lymphocyte leukemia with

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Ti (WT31)-negative, CD3-positive, large granular lymphocyte leukemia with nonspecific cytotoxicity.

nonspecific cytotoxicity Ti (WT31)-negative, CD3-positive, large granular lymphocyte leukemia with