10例心脏淋巴瘤患者的临床资料分析

A clinical analysis of 10 cases with cardiac lymphoma

目的分析心脏淋巴瘤的发病情况、临床特征、治疗效果及预后。方法收集2000年1月至2016年6月期间确诊并有心脏累及的10例淋巴瘤患者的资料，对患者的一般资料、临床表现、病理诊断、实验室检查、心脏累及方式、心脏并发症、治疗方式、疗效及预后进行分析。结果3918例淋巴瘤患者中，心脏累及者10例，其中原发性心脏淋巴瘤（PCL）1例（主要累及左右心房，以心肌内多发结节包块为主），继发性心脏淋巴瘤（SCL）9例（主要为心包包块，其中出现心包积液5例，心肌肿块2例）。男性6例，女性4例，中位年龄55（19～88）岁，主要临床表现为呼吸困难7例，胸痛5例，乏力、水肿各2例。病理类型包括弥漫大B细胞淋巴瘤（DLBCL）7例，T淋巴母细胞淋巴瘤、霍奇金淋巴瘤、Burkitt淋巴瘤各1例。心脏并发症包括充血性心力衰竭7例，心律失常4例（主要为窦性心动过速、心房颤动和房室传导阻滞）。除1例高龄、一般状况差未接受治疗外，其余9例患者均接受治疗（单纯化疗4例，化疗联合放疗5例）。中位随访时间为9（1～28）个月。1例PCL患者化疗后获部分缓解（PR），无进展生存（PFS）期为6个月，总生存（OS）期为21个月。SCL患者中6例起病累及心脏者，治疗后1例获完全缓解，5例获PR，中位PFS期为5个月，中位OS期为19个月；3例病情进展累及心脏者，2例治疗后获PR，1例未治疗者死亡，中位PFS期为4个月，OS因数据截尾，未能获得。结论心脏淋巴瘤为少见类型，DLBCL为最常见类型，呼吸困难、胸痛为最常见临床表现，并易出现充血性心力衰竭和心律失常，治疗以系统化疗为主，总体预后差。

Objective To analyze the morbidity, clinical characteristics, therapeutic outcomes and prognosis of cardiac lymphoma. Methods Individual patient data were obtained from pathology defined l0 cases of cardiac lymphoma from Jan 2000 to Jun 2016. The patient＇ s general information, clinical manifestation, pathological diagnosis, laboratory examination, cardiac involvement feature, cardiac complications, treatment, therapeutic effect and prognosis were analyzed. Results Of 3 918 cases of lymphoma patients, 10 cases of cardiac involvement were identified, including primary cardiac lymphoma （PCL） in 1 case, secondary cardiac lymphoma （SCL） in 9 cases. Of the l0 patients in our analysis, the male-to-female ratio was 3：2, with a median age of 55 （19-88） years old. The most presenting complaints were dyspnea in 7 cases, followed by chest pain in 5 cases, fatigue in 2 patients and edema in 2 cases. Pathological types included diffuse large B cell lymphoma （DLBCL） in 7 cases, T cell lymphoma （T- LBL） in 1 case, Hodgkin＇ s lymphoma （HL） in 1 case, and Burkitt lymphoma （BL） in 1 case. The sites of the heart affected by lyrnphoma in the PCL patient were right and left atriums with multiple nodules; and for SCL, the sites were mainly pericardium associated with a pericardial effusion in 5 cases, a pericardial mass in 2 cases. Congestive heart failure affects 7 patients and cardiac arrhythmias were identified in 4cases mainly sinus tachycardia, atrial fibrillation and atrioventricular block. Except one untreated because of old age and poor performance, the rest of 9 patients were treated by either chemotherapy in 4 cases or chemotherapy combined radiotherapy （including the extracardiac sites） in 5 patients. With the median follow-up of 9 months, the one PCL patient achieved partial response （PR）, progress free survival （PFS） for 6 months and the overall survival （OS） for 21 months; in the cohort of 6 SCL patients cardiac involved at diagnosis, complete response （CR） was achieved in 1 case （16.7%）, PR in 3 cases, progressing disease （PD） in 2 cases, with the median PFS for 5 months and the median OS for 19 months; and for the other 3 SCL patients cardiac involved at progression, PR was achieved in 2 case and death in 1 case, with the median PFS for 4 months and the median OS unavailable because of censored data. Conclusion Cardiac lymphoma represents a rare subset of lymphoma, the most common type is DLBCL, and the main clinical manifestations are dyspnea and chest pain, always combined by arrhythmia and congestive heart failure. The main therapeutic regimen for cardiac lymphoma includes combined chemotherapy and the prognosis for patients with either PCL or SCL is usually poor.