Patients with large granular lymphocytic leukemia (LGLL) frequently present with neutropenia. When present, anemia is usually accompanied by neutropenia and/or thrombocytopenia and isolated anemia is uncommon. We evaluated a cohort of 244 LGLL patients spanning 15 years and herein report the clinicopathologic features of 34 (14%) with isolated anemia. The patients with isolated anemia showed a significantly male predominance (p = 0.001), a lower level of hemoglobulin (p < 0.0001) and higher MCV (p = 0.017) and were less likely to have rheumatoid arthritis (p = 0.023) compared to the remaining 210 patients. Of the 34 LGLL patients with isolated anemia, 13 (38%) presented with pure red cell aplasia (PRCA), markedly decreased reticulocyte count and erythroid precursors, and more transfusion-dependence when compared to non-PRCA patients. There was no other significant clinicopathologic difference between PRCA and non-PRCA patients. 32 patients were followed for a median duration of 51 months (6–199). 24 patients were treated (11/11 PRCA and 13/21 non-PRCA patients, p < 0.02). The overall response rate to first-line therapy was 83% [8/11 (72.7%) for PRCA, 12/13 (92.3%) for non-PRCA], including 14 showing complete response and 6 showing partial response with a median response duration of 48 months (12–129). Half of non-PRCA patients who were observed experienced progressive anemia. During follow-up, no patients developed neutropenia; however, 5/27 (18.5%) patients developed thrombocytopenia. No significant difference in overall survival was noted between PRCA and non-PRCA patients. In summary, this study demonstrates the unique features of LGLL with isolated anemia and underscores the importance of recognizing LGLL as a potential cause of isolated anemia, which may benefit from disease-specific treatment. LGLL patients with PRCA were more likely to require treatment but demonstrated similar clinicopathologic features, therapeutic responses, and overall survival compared to isolated anemia without PRCA, suggesting PRCA and non-PRCA of T-LGLL belong to a common disease spectrum.
大颗粒淋巴细胞白血病（LGLL）患者常伴有中性粒细胞减少。若出现贫血，通常同时存在中性粒细胞减少和/或血小板减少，单纯性贫血较为少见。我们对15年间244例LGLL患者队列进行了评估，本文报告其中34例（14%）单纯性贫血患者的临床病理特征。与其余210例患者相比，单纯性贫血患者男性占比显著更高（p=0.001）、血红蛋白水平更低（p<0.0001）、平均红细胞体积更大（p=0.017），且类风湿关节炎患病率更低（p=0.023）。在34例单纯性贫血LGLL患者中，13例（38%）表现为纯红细胞再生障碍（PRCA），与非PRCA患者相比，其网织红细胞计数和红系前体细胞显著降低，且输血依赖程度更高。PRCA与非PRCA患者之间未发现其他显著临床病理差异。对32例患者进行了中位51个月（6-199个月）的随访，其中24例接受治疗（PRCA组11/11，非PRCA组13/21，p<0.02）。一线治疗总缓解率为83%[PRCA组8/11（72.7%），非PRCA组12/13（92.3%）]，包括14例完全缓解和6例部分缓解，中位缓解持续时间为48个月（12-129个月）。未接受治疗的非PRCA患者中有半数出现进行性贫血。随访期间无患者发生中性粒细胞减少，但5/27（18.5%）患者出现血小板减少。PRCA与非PRCA患者的总生存期无显著差异。本研究揭示了伴单纯性贫血的LGLL独特特征，强调需认识LGLL作为单纯性贫血潜在病因的重要性，此类患者可能从疾病特异性治疗中获益。伴PRCA的LGLL患者更需治疗，但其临床病理特征、治疗反应和总生存期与不伴PRCA的单纯性贫血患者相似，提示T-LGLL伴或不伴PRCA可能属于同一疾病谱系。
Isolated anemia in patients with large granular lymphocytic leukemia (LGLL)
肿瘤(癌症)患者之家