Oshimi K, Yamada O, Kaneko T, Nishinarita S, Iizuka Y, Urabe A, Inamori T, Asano S, Takahashi S, Hattori M
Department of Hematology, Tokyo Women's Medical College, Japan.
Leukemia. 1993 Jun;7(6):782-8.
The hematological and immunological findings and clinical courses of 33 patients (13 male, 20 female; median age at presentation, 60 years) with granular lymphocyte-proliferative disorders (GLPD) are presented. Based on the surface phenotypes of peripheral blood granular lymphocytes (GL), the GLPD were divided into CD3+ T cell-lineage GLPD (T-GLPD) and CD3- CD16+ natural killer (NK) cell-lineage GLPD (NK-GLPD). Twenty-one patients had T-GLPD, and 12 had NK-GLPD. One patient with T-GLPD and two patients with NK-GLPD had progressive clinical courses and died of the disease despite receiving combination chemotherapy. Twelve patients with T-GLPD were found to have severe anemia at presentation or during the course of the disease; four of them fulfilled the diagnostic criteria of pure red cell aplasia, and the others had closely related conditions. Six of these 12 patients were treated with cyclophosphamide, and all responded to the treatment. In 16 patients, the clinical course was stable, and spontaneous regression was observed in two patients. Since some of the patients with NK-GLPD had stable clinical courses while some had progressive clinical courses, clinical findings in these two groups were compared. We found, taking into consideration our cases and those reviewed in the literature, that age less than 40 years, fever, lymph node swelling, hepatosplenomegaly, and GL with CD16(Leu-11)-CD56+CD57- phenotype and low or absent antibody-dependent cellular cytotoxicity seemed to be predictors of a progressive clinical course.
本文报告了33例颗粒淋巴细胞增殖性疾病（GLPD）患者（13例男性，20例女性；就诊时中位年龄60岁）的血液学和免疫学检查结果及临床病程。根据外周血颗粒淋巴细胞（GL）的表面表型，将GLPD分为CD3⁺T细胞系GLPD（T-GLPD）和CD3⁻CD16⁺自然杀伤（NK）细胞系GLPD（NK-GLPD）。21例患者为T-GLPD，12例为NK-GLPD。1例T-GLPD患者和2例NK-GLPD患者临床病程呈进行性，尽管接受了联合化疗仍死于该疾病。12例T-GLPD患者在就诊时或病程中出现严重贫血；其中4例符合纯红细胞再生障碍的诊断标准，其余患者有密切相关疾病。这12例患者中的6例接受了环磷酰胺治疗，所有患者对治疗均有反应。16例患者临床病程稳定，2例患者出现自发缓解。由于部分NK-GLPD患者临床病程稳定而部分呈进行性，因此对这两组患者的临床特征进行了比较。综合考虑我们的病例及文献复习结果，我们发现年龄小于40岁、发热、淋巴结肿大、肝脾肿大以及具有CD16（Leu-11）-CD56⁺CD57⁻表型且抗体依赖性细胞毒性低或缺乏的GL似乎是临床病程呈进行性的预测因素。
