慢性粒-单核细胞白血病合并结外非霍奇金淋巴瘤的临床特征分析

doi:10.16150/j.1671-2870.2018.01.014

摘要/Abstract

摘要： 目的：探讨慢性粒-单核细胞白血病(chronic myelomonocytic leukemia, CMML)合并结外非霍奇金淋巴瘤（non-Hodgkin's lymphoma,NHL）患者的临床特征及其鉴别诊断。方法：回顾性分析2例CMML合并结外NHL患者的临床资料、影像学检查、病理形态学及免疫组织化学（免疫组化）标志特征,并复习国内外相关文献进行探讨。结果：2例CMML合并结外NHL患者均为老年女性,因乏力或出现相应部位肿瘤侵犯症状就诊。例1患者诊断为CMML后发生NHL,例2患者则同时诊断为CMML和NHL。实验室检查示,2例患者的外周血白细胞计数增高,以单核细胞为主;乳酸脱氢酶升高;骨髓增生活跃伴有病态造血;细胞免疫表型为CD14⁺CD64⁺;染色体荧光原位杂交分析BCR-ABL为阴性(-)。PET/CT 检查显示 2例患者均未发现骨髓高代谢灶,例1患者表现为小肠异常高代谢,最大标准化摄取值（maximum standard uptake values,SUVmax）值为12.60;例2患者表现为肺内异常高代谢,SUVmax值为6.72。病理检查提示2例患者均为B细胞来源的NHL;免疫组化检查结果提示,LCA、CD20、CD79a、Bcl-6、Ki-67均为阳性,CD5、CD10、CD3、CylinD1、CD56、CD23、CD21阴性。结论：CMML合并结外NHL罕见,患者缺乏特异性的临床表现,而结合实验室、影像学检查及组织病理、免疫组化检查进行分析,可提高其诊断的准确率。

关键词: 慢性粒-单核细胞白血病, 非霍奇金淋巴瘤, 诊断

Abstract: Objective: To investigate the clinical characteristics and differential diagnosis of chronic myelomonocytic leukemia (CMML) accompanied with non-Hodgkin 's lymphoma (NHL). Methods: The clinical data, imaging findings, pathomorphological features and immunohistochemical markers of two patients with CMML accompanied with NHL were retrospectively analyzed, and related literatures were reviewed. Results: The 2 cases were elderly women presented with fatigue or symptoms of tumor invasion at corresponding sites. Case 1 was diagnosed with CMML and thereafter having NHL diagnosed, and case 2 was diagnosed with CMML and NHL simultaneously. Laboratory examination showed that peripheral white blood cells were increased,especially mononuclear cells, and lactate dehydrogenase was also increased. Bone marrow smears showed active proliferation with morbid hematopoiesis. On immunophenotyping, the bone marrow cells often expressed CD14⁺CD64⁺. Analysis of chromosome fluorescence in situ hybridization showed that BCR-ABL fusion gene were negative. PET/CT examination displayed that high metabolic lesions in bone marrow were not found in the 2 cases, but showed hypermetabolic small intestinal lesions in case 1 and lung lesions in case 2. SUVmax value were 12.60 and 6.72, respectively. Pathological examination suggested that these 2 patients were B-cell-derived NHL. Immunohistochemical study showed that LCA, CD20, CD79a, Bcl-6 and Ki-67 were positive, CD5, CD10, CD3, CylinD1, CD56, CD23 and CD21 were negative. Conclusions: CMML accompanied with NHL is rare and lacks specific clinical manifestation. With the laboratory findings combined with imaging, histopathology and immunohistochemical examination, the diagnostic accuracy could be improved.

Key words: Chronic myelomonocytic leukemia, Non-Hodgkin 's lymphoma, Diagnosis

中图分类号:

R733.4

李佳明, 张苏江, 王莹, 严泽莹, 刘之茵, 孙海敏, 陈玉宝, 陈钰, 罗方秀, 孙静. 慢性粒-单核细胞白血病合并结外非霍奇金淋巴瘤的临床特征分析[J]. 诊断学理论与实践, 2018, 17(01): 76-81.

LI Jiaming, ZHANG Sujiang, WANG Ying, YAN Zeying, LIU Zhiyin, SUN Haimin, CHEN Yubao, CHEN Yu, LUO Fangxiu, SUN Jing. Chronic myelomonocytic leukemia accompanied with extranodal lymphoma : a clinical feature analysis[J]. Journal of Diagnostics Concepts & Practice, 2018, 17(01): 76-81.

参考文献

[1] Arber DA, Orazi A, Hasserjian R, et al.The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia[J]. Blood,2016, 127(20):2391-2405.
[2] Itzykson R, Duchmann M, Lucas N, et al.CMML: Clinical and molecular aspects[J]. Int J Hematol,2017,105(6):711-719.
[3] Harada Y, Harada H. Rinsho Ketsueki.Chronic myelomonocytic leukemia(CMML): recent advances in molecular pathogenesis and treatment[J]. Rinsho Ketsueki,2016,57(2):147-155.
[4] Bacher U, Haferlach T, Schnittger S, et al.Recent advances in diagnosis, molecular pathology and therapy of chronic myelomonocytic leukaemia[J]. Br J Haematol,2011,153(2):149-167.
[5] Such E, Germing U, Malcovati L, et al.Development and validation of a prognostic scoring system for patients with chronic myelomonocytic leukemia[J]. Blood,2013,121(15):3005-3015.
[6] Elena C, Gallì A, Such E, et al.Integrating clinical features and genetic lesions in the risk assessment of patients with chronic myelomonocytic leukemia[J]. Blood,2016,128(10):1408-1417.
[7] Koss MN.Malignant and benign lymphoid lesions of the lung[J]. Ann Diagn Pathol,2004,8(3):167-187.
[8] Ahmed S, Siddiqui AK, Rai KR.Low-grade B-cell bronchial associated lymphoid tissue(BALT) lymphoma[J]. Cancer Invest,2002,20(7-8):1059-1068.
[9] Wei S, Li X, Qiu X, et al.Primary lung lymphoma involving the superior vena cava[J]. World J Surg Oncol,2012,10:131.
[10] Hans CP, Weisenburger DD, Greiner TC, et al.Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray[J]. Blood,2004,103(1):275-282.
[11] 陈光祥, 蔡亮, 张伟, 等. ¹⁸F-FDG PET/CT 在淋巴瘤结外侵犯中的诊断价值[J]. 放射学实践,2013,28(1):95-98.
[12] Cabanillas F, Rivera N, Pardo WI.Indolent Lymphomas That Present With Clinically Aggressive Features: A Subset of Low-Grade Lymphomas With a Behavior Inconsistent With the Histologic Diagnosis[J]. Clin Lymphoma Myeloma Leuk,2016,16(10):550-557.
[13] 霍真, 李霁, 刘鸿瑞, 等. 北京协和医院对18年间病理诊断肺淋巴瘤患者的回顾分析[J]. 中华肺部疾病杂志,2016,9(6):600-603.
[14] 关炜, 王全师, 吴湖炳, 等. 原发性肠淋巴瘤的18F-FDG PET/CT影像学表现[J]. 南方医科大学学报,2016,36(9):1175-1180.
[15] Tamayo P, Martín A, Díaz L, et al.¹⁸F-FDG PET/CT in the clinical management of patients with lymphoma[J]. Rev Esp Med Nucl Imagen Mol,2017,36(5):312-321.
[16] Saillard C, Guermouche H, Derrieux C, et al.Response to 5-azacytidine in a patient with TET2-mutated angioimmunoblastic T-cell lymphoma and chronic myelomonocytic leukaemia preceded by an EBV-positive large B-cell lymphoma[J]. Hematol Oncol,2017,35(4):864-868.
[17] Grignano E, Mekinian A, Braun T, et al.Autoimmune and inflammatory diseases associated with chronic myelomonocytic leukemia: A series of 26 cases and litera-ture review[J]. Leuk Res,2016,47:136-141.
[18] Mathew RA, Bennett JM, Liu JJ, et al.Cutaneous manifestations in CMML: Indication of disease acceleration or transformation to AML and review of the literature[J]. Leuk Res,2012,36(1):72-80.
[19] 王媛媛, 沈金辉, 王少洪. 传染性单核细胞增多症淋巴结病临床病理观察[J]. 诊断病理学杂志,2015,22(10):614-616.
[20] Toriihara A, Nakajima R, Arai A, et al.Pathogenesis and FDG-PET/CT findings of Epstein-Barr virus-related lymphoid neoplasms[J]. Ann Nucl Med,2017,31(6):425-436.
[21] 于亚平, 宋萍, 安志明, 等. 成人非典型传染性单核细胞增多症临床病理特点[J]. 中国实验血液学杂志,2016,24(6):1873-1878.