[Hematopathologic features of T-cell large granular lymphocytic leukemia].
OBJECTIVE
To explore the hematopathologic features of T-cell large granular lymphocytic leukemia (T-LGLL).


METHODS
A retrospective analysis of the clinical presentation, bone marrow morphology, immunophenotyping and T-cell receptor gene rearrangement status were performed in 19 patients with T-LGLL.


RESULTS
Of 19 patients, the most frequent hematological abnormalities were anemia and neutropenia (16/19 and 17/19 patients, respectively). Large granular lymphocytes (LGLs) were observed in 17 of 19 peripheral blood smears and 15 of 19 bone marrow aspirate specimens. Lymphocytosis (> 0.2) was present in 17 of 19 patients in their bone marrow aspirate specimens. Bone marrow biopsy specimens revealed lymphocytosis in 16 cases, with a mild to moderate increase of lymphocytes observed in 12 cases (12/16). The pattern of lymphoid distribution was interstitial in bone marrow sections. Intravascular distribution was seen in 8 cases. Lymphoid nodules were present in 4 cases. Flow cytometery showed an immunophenotype of CD3(+) CD4(-) CD8(+) CD56(-) CD57(+) of the tumor cells in 13 cases. Of the other 6 cases, the immunophenotypes included CD8(-) (1 case), CD56(+) (2 cases) and CD57(-) (3 cases). Immunohistochemistry showed CD3+ (10/10), CD57+ (3/3), CD8+ (6/7), TIA-1+ (6/7), granzyme B+ (4/7), perforin + (1/7), CD4- (4/4) and CD56- (9/9). Clonal T-cell receptor γ gene rearrangement by PCR was detected in 12 cases (12/17).


CONCLUSIONS
Hematopathologic features of most T-LGLL are distinct. Morphologic, immunophenotypic and molecular analysis of both peripheral blood and bone marrow specimens are essential and complementary in the diagnosis and differential diagnosis of T-LGLL.
Department of Pathology, Hospital of Blood Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin 300020, China.
En-bin
EB
Liu
Hui-shu
HS
Chen
Pei-hong
PH
Zhang
Zhan-qi
ZQ
Li
Qi
Q
Sun
Qing-ying
QY
Yang
Li-huan
LH
Fang
Fu-jun
FJ
Sun
D000328:Adult; D000368:Aged; D000740:Anemia / Q000378:metabolism / Q000473:pathology*; D001853:Bone Marrow / Q000473:pathology*; D017252:CD3 Complex / Q000378:metabolism; D018959:CD57 Antigens / Q000378:metabolism; D016827:CD8 Antigens / Q000378:metabolism; D003937:Diagnosis, Differential; D005260:Female; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D053804:Granzymes / Q000378:metabolism; D006801:Humans; D016130:Immunophenotyping; D054066:Leukemia, Large Granular Lymphocytic / Q000378:metabolism / Q000473:pathology*; D008218:Lymphocytosis / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009503:Neutropenia / Q000378:metabolism / Q000473:pathology*; D039102:Poly(A)-Binding Proteins / Q000378:metabolism; D012189:Retrospective Studies; D000074019:T-Cell Intracellular Antigen-1
D017252:CD3 Complex; D018959:CD57 Antigens; D016827:CD8 Antigens; D039102:Poly(A)-Binding Proteins; D000074019:T-Cell Intracellular Antigen-1; C071558:TIA1 protein, human; D053804:Granzymes