CAJ | 학술논문

目的分析2例胸腺瘤/T-大颗粒淋巴细胞白血病(T-LGLL)合并纯红细胞再生障碍(PRCA)患者的临床及实验室特征,提高对该类疾病的认识并了解淋巴细胞在疾病中的作用.方法对2例胸腺瘤/T-LGLL合并PRCA患者的病例资料进行分析,并复习相关文献.结果例1,男,63岁,入院时HGB 54 g/L,网织红细胞绝对值9.8×109/L;例2,女,52岁,入院时HGB 79 g/L,网织红细胞绝对值30.8×109/L.临床表现均以贫血为主,起病缓慢,于外院确诊为胸腺瘤合并PRCA,行胸腺瘤切除术.术后血常规无改善.复查外周血涂片中大颗粒淋巴细胞增多,免疫表型检查示淋巴细胞表达CD3+ CD4-CD8+ CD57+,TCR基因重排阳性.确诊为胸腺瘤/T-LGLL合并PRCA,胸腺瘤切除术后.例l对环孢素、环磷酰胺、泼尼松、甲氨蝶呤等均无治疗反应,例2经环孢素治疗血常规恢复正常.结论胸腺瘤/T-LGLL可引起继发性PRCA,淋巴细胞增殖在其中可能发挥重要作用.
목적 분석2례흉선류/T-대과립림파세포백혈병(T-LGLL)합병순홍세포재생장애(PRCA)환자적림상급실험실특정,제고대해류질병적인식병료해림파세포재질병중적작용.방법 대2례흉선류/T-LGLL합병PRCA환자적병례자료진행분석,병복습상관문헌.결과 례1,남,63세,입원시HGB 54 g/L,망직홍세포절대치9.8×109/L;례2,녀,52세,입원시HGB 79 g/L,망직홍세포절대치30.8×109/L.림상표현균이빈혈위주,기병완만,우외원학진위흉선류합병PRCA,행흉선류절제술.술후혈상규무개선.복사외주혈도편중대과립림파세포증다,면역표형검사시림파세포표체CD3+ CD4-CD8+ CD57+,TCR기인중배양성.학진위흉선류/T-LGLL합병PRCA,흉선류절제술후.례l대배포소、배린선알、발니송、갑안접령등균무치료반응,례2경배포소치료혈상규회복정상.결론 흉선류/T-LGLL가인기계발성PRCA,림파세포증식재기중가능발휘중요작용.
Objective To investigate the clinical and laboratory features ot 2 cases of pure red cell aplasia (PRCA) with thymoma/T-cell large granular lymphocyte leukemia (T-LGLL),and to improve the recognition of the disease and the role of lymphocyte in its mechanism.Methods Two cases of PRCA with thymoma/T-LGLL were reported and the related literatures were reviewed.Results Case 1 was a 63-years old male with hemoglobin level of 54 g/L at admission.Case 2 was a 52-years old female with hemoglobin level of 79 g/L at admission.They were both diagnosed as PRCA with thymoma before admission to our hospital and had no benefit from their thymectomy.Further examinations in our hospital showed that CD3 + CD4-CD8 + CD57 + large granular lymphocytes amplified with clonal TCR rearrangment in their peripheral blood.The diagnosis of PRCA with thymoma/T-LGLL was clarified.Case 1 did not respond to any of the frontline therapies while case 2 responded completely to cyclosporine.Conclusion Both thymoma and T-LGLL could be the cause of secondary PRCA,lymphocyte proliferation may play critical role in the pathogenesis.