Laboratory findings and clinical courses of 33 patients with granular lymphocyte-proliferative disorders.
Laboratory findings and clinical courses of 33 patients with granular lymphocyte-proliferative disorders.
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33 例颗粒淋巴细胞增殖性疾病患者的实验室检查结果和临床过程。
作者：
K. Oshimi;O. Yamada;T. Kaneko;Susumu Nishinarita;Yoshikazu Iizuka;Akio Urabe;T. Inamori;Shigetaka Asano;Satoshi Takahashi;M. Hattori
33例（13男，20女;中位年龄在介绍，60岁）与颗粒淋巴细胞增殖性疾病（GLPD）的血液学和免疫学的结果和临床过程。根据外周血颗粒淋巴细胞（GL）的表型，将GLPD分为CD 3 + T细胞系GLPD（T-GLPD）和CD 3-CD 16+自然杀伤（NK）细胞系GLPD（NK-GLPD）。21例患者患有T-GLPD，12例患有NK-GLPD。1例T-GLPD患者和2例NK-GLPD患者尽管接受了联合化疗，但仍有进展性临床病程并死于疾病。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性贫血的诊断标准，其他患者有密切相关的疾病。这12例患者中有6例接受环磷酰胺治疗，均对治疗有反应。16例患者的临床病程稳定，2例患者观察到自发消退。由于部分NK-GLPD患者的临床病程稳定，而部分患者的临床病程进展，因此比较了这两组患者的临床结果。我们发现，考虑到我们的病例和文献中回顾的病例，年龄小于40岁、发热、淋巴结肿胀、肝脾肿大和GL伴CD 16（Leu-11）-CD 56 + CD 57-表型和低或无抗体依赖性细胞毒性似乎是进展性临床病程的预测因子。
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.