Immunophenotypic Profile In T Large Granular Lymphocyte Leukemia Patients

Objective:T-cell large granular lymphocytic leukemia(T-LGLL)is a rare cytotoxic T lymphocyte proliferative tumor.The World Health Organization has positioned it as a heterogeneous disease with continuous clonal proliferation of large lymphocytes.T-LGLL manifests as hematopoietic failure or one or several cell lines with immune-mediated destruction,including red blood cells,neutrophils and platelets.Patients are usually over 50 years old,have repeated bacterial infections,occasionally splenomegaly,rheumatoid arthritis or other lymphatic proliferative diseases.The key is how to accurately diagnose T-LGLL through relevant laboratory tests.Immunophenotypic and clonal studies,combined with patients’ clinical manifestations,are crucial to understanding this type of disease.Methods:In present study,we selected 195 patients with confirmed diagnosed T-cell large granular lymphocytic leukemia and clonality evidence.Peripheral blood and/or bone marrow were ectracted,the initial symptoms,peripheral blood cells count at the onset,the number of circulating T-LGL are all summarized for analysis.Based on flow cytometry technolog,we analyzed the immunophenotypes and TCR-vβ.And we used polymerase chain reaction(PCR)method to detected the TCR gene rearrangement.The objective is to explore the correlation between the clinical characteristics of T-LGLL and the number and immunophenotypic characteristics of LGL in circulation.Results:The number of circulating LGLs was 0.13-70.23 ×109/L in 195 T-LGLL patients,and the median was 2.10×109/L.There were 97 patients with simple anemia,accounting for 49.7%of all patients,13 patients with simple agranulocytosis,accounting for 6.67%,and 45 patients with two-line cytopenia,accounting for 23.1%of all patients;9 patients with pancytopenia,accounting for 4.6%of all patients;28 patients with lymphocytosis,accounting for 14.4%of all patients;2 patients with thrombocytopenia,accounting for 1.0%of all cases;1 patient without abnormal blood count,accounting for 0.5%of all patients.There were 57 patients who had no indications for treatment at the time of consultation,and 138 patients required clinical intervention.Flow cytometry showed that there were 163 cases of TCRαβ type,accounting for about 83.59%of the total patients,32 patients of TCRγδ type,accounting for about 16.41%;159 cases of CD8+T-LGLL patients among the 195 patients,accounting for 81.54%,14 cases of CD4+T-LGLL,accounting for 7.18%,11 patients of CD4 and CD8 double positive,accounting for 5.64%,11 cases of CD4 and CD8 double negative,accounting for 5.64%.There were 186 patients with CD57 expression,accounting for 95.38%,and 9 patients without CD57 expression,accounting for 4.62%.There were 175 patients with weakened or missing CD5 expression,accounting for 89.74%,143 patients with weakened or missing CD7 expression,accounting for 73.33%,and 78 patients with weakened or missing CD2 expression,accounting for 40.00%.Through logistic regression analysis,the number of LGL was slightly negatively correlated with whether or not the treatment against was reached(P=0.004,OR=0.918),while expression of TCRγδ was not related to it(P=0.164).The weakened/absent expression of CD5 is positively correlated with whether or not the treatment against was reached(P=0.022,OR=3.830),and the weakened/absent expression of CD2 and CD7 have nothing to do with it.Anemia and agranulocytosis were positively correlated with the accompanying expression of CD56((P=0.000,OR=47.269)vs(P=0.025,OR=10.733)Conclusion:Our results showed the T-LGLL patients with CD8+TCRαβ type are most common in this study.Expression of TCRγδ was not associated with achievement of treatment against.Patients with the weakening of CD5 expression is positively correlated with the achievement of treatment against,well the number of LGL in the circulation is negatively correlated with it.Both anemia and agranulocytosis are positively correlated with the accompanying expression of CD56.