Clinical and laboratory features of T-cell prolymphocytic leukemia in China
ZHANG Yan-ru, QI Jun-yuan, LIU Hui-min, LIU Wei, HUANG Wen-yang, DENG Shu-hui, YI Shu-hua
Published 2013-10-15
Cite as Chin J Hematol, 2013,34(10): 839-843. DOI: 10.3760/cma.j.issn.0253-2727.2013.10.004
Abstract
Objective To investigate the clinical and laboratory characteristics and survival of Chinese patients with T-cell prolymphocytic leukemia (T-PLL).Method Eleven patients with T-PLL admitted in our hospital from Jan 2006 to Oct 2012 were retrospectively analyzed.Results Of the 11 patients,nine were males and two females,with the median age of 56.0(19-69) years old.All the patients,except for three,presented with leukocytosis.The incidence of hyperleukocytosis (1/11) was less frequent than that in the British series (75%) (P =0.000).Lymphocyte counts in peripheral blood were increased in 9 of the 11 patients with the median absolute lymphocyte count (ALC) of 17.22 (0.58-148.83)× 109/L.Superficial lymphadenopathy and splenomegaly were the most common physical signs.It was common that serum lactate dehydrogenase (LDH) and beta 2 microglobulin (β2-MG) were higher than normal level.All cases were positive for CD2/CD3/CD5/TCRαβ,negative for CD1a/HLA-DR and TdT,and most of them were strong positive for CD7 expression.By chromosome analyses,most cases (9/10) have normal chromosome.This rate is significantly higher than that of the British and American series (3% and 25%,respectively) (P =0.000,P =0.001).The 14qll abnormality and trisomy 8q,which are common among Western cases,were not observed in any of our cases.With a median follow-up of 23.0 months,three patients died.Two year progress free survival (PFS) and overall survival (OS) were 53.3% and 50%,respectively.There were 3 patients with PFS over a number of years,whether it should be considered as the T-chronic lymphocytic leukemia (T-CLL) is worthy of further studies.Conclusion The common clinical manifestations of T-PLL patients were increased lymphocyte counts and lymphadenopathy as well as splenomegaly.And most cases have high level of blood LDH and β2-MG and normal chromosome karyotype.
Key words:
Leukemia, prolymphocytic, T-cell; Clinical features; Cytogenetics
Contributor Information
ZHANG Yan-ru
Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China
QI Jun-yuan
Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China
LIU Hui-min
Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China
LIU Wei
Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China
HUANG Wen-yang
Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China
DENG Shu-hui
Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China
YI Shu-hua
Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China