Clinical analysis of 52 patients with granular lymphocyte proliferative disorder (GLPD) showed frequent anemia in indolent T-cell GLPD in Japan.
We present here clinical and hematological findings of 52 cases of granular lymphocyte-proliferative disorder (GLPD), which contained 35 indolent T-cell lineage granular lymphocyte-proliferative disorder (T-GLPD), two atypical T-GLPD, 12 chronic NK-cell lymphocytosis (CNKL), and three aggressive NK-cell leukemia (ANKL). The median period of follow up was 24 months. Hemoglobin level <8.0 g/dL was recognized in 21 cases of indolent T-GLPD (60%), among which 15 patients met the criteria of pure red cell aplasia. Neutrophil counts <500/microL occurred only in two cases of T-GLPD (6%). Although the median age and male-to-female distribution were similar, very frequent anemia and rare neutrocytopenia in indolent T-GLPD in the present study keenly contrasted with previous reports. CD56 was positive in three of 29 indolent T-GLPD cases with CD4-CD8+ phenotype, in three of four CD4+CD8-, and in none of two CD4-CD8- cases. Therefore, although two atypical T-GLPD cases were CD56-positive, CD56 should not be a specific marker for aggressive T-GLPD. All CNKL patients had a chronic course with a stable granular lymphocyte count. All three ANKL patients presented high fever and hepatosplenomegaly, barely responded to chemotherapies and died within 6 months. The present analysis of 52 cases of GLPD in Japan showed that Japanese and Western cases of indolent T-GLPD clearly differ in their hematological complications.
Kawahara
Shimpei
S
Division of Hematology, Department of Internal Medicine, Juntendo University School of Medicine, Tokyo, Japan.
Sasaki
Makoto
M
Isobe
Yasushi
Y
Ando
Jun
J
Noguchi
Masaaki
M
Koike
Michiaki
M
Hirano
Takao
T
Oshimi
Kazuo
K
Sugimoto
Koichi
K
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000740:Anemia / Q000453:epidemiology*; D015703:Antigens, CD / Q000097:blood; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007564:Japan; D007621:Karyotyping; D007938:Leukemia / Q000453:epidemiology; D007958:Leukocyte Count; D008232:Lymphoproliferative Disorders / Q000097:blood* / Q000188:drug therapy / Q000235:genetics / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D013601:T-Lymphocytes / Q000276:immunology*; D055815:Young Adult
D015703:Antigens, CD; D007166:Immunosuppressive Agents