Palomera Bernal L, García Díez I
Servicio de Hematología y Hemoterapia, Hospital Comarcal Infanta Margarita, Cabra, Córdoba.
Med Clin (Barc). 1990 Feb 24;94(7):262-5.
We report a patient with nonregenerative anemia and neutropenia associated with an increment of circulating large granular lymphocytes (LGL). The anemia was secondary to the absence of erythroid precursors in the bone marrow or pure red blood cell aplasia associated with moderate lymphoid infiltration by LGL. Myeloid (CFU-GM) or erythroid (CFU-E, BFU-E) precursors were not detected by bone marrow culture. A high number of T colonies was found. The lymphoid population had a cytotoxic/suppressor phenotype (CD2+, CD4-, CD8+). Virologic studies (including search for HTLV1) were carried out with negative results. A clonal origin was demonstrated by DNA analysis with probes of those genes encoding T receptor (TRc). After cytostatic therapy with cyclophosphamide and low doses of prednisone a clinical and laboratory remission was achieved. We review the literature, with a discussion of the clinical, phenotypic and molecular features of this disease as well as its response to therapy.
我们报告了一名患有非再生性贫血和中性粒细胞减少症的患者,其循环大颗粒淋巴细胞(LGL)增多。贫血继发于骨髓中缺乏红系前体细胞或与LGL中度淋巴样浸润相关的纯红细胞再生障碍。骨髓培养未检测到髓系(CFU-GM)或红系(CFU-E、BFU-E)前体细胞。发现大量T集落。淋巴样细胞群具有细胞毒性/抑制表型(CD2+、CD4-、CD8+)。进行了病毒学研究(包括检测HTLV1),结果为阴性。用编码T受体(TRc)的基因探针进行DNA分析,证实了克隆起源。在用环磷酰胺和低剂量泼尼松进行细胞抑制治疗后,实现了临床和实验室缓解。我们回顾了文献,讨论了该疾病的临床、表型和分子特征及其对治疗的反应。