伴血象异常的脾边缘区淋巴瘤的临床特点  

Clinical Characteristics of Splenic Marginal Zone Lymphoma with Abnormal Complete Blood Count

作　　者：杨申淼[1] 江倩[1] 江滨[1] 陈定宝[2] 王婧[1] 江浩[1] 路瑾[1] 卢锡京[1] 鲍立[1] 石红霞[1] 刘艳荣[1] 黄晓军[1] 

机构地区：[1]北京大学人民医院、北京大学血液病研究所,北京100044 [2]北京大学人民医院病理科,北京100044

出　　处：《中国实验血液学杂志》2013年第1期87-94,共8页Journal of Experimental Hematology

摘　　要：本文探讨伴有血细胞计数异常的脾边缘区淋巴瘤(SMZL)的临床特点。回顾性地分析了19例因血细胞计数异常就诊并被确诊为SMZL患者的资料,其中7例根据脾脏病理确诊,12例不能接受脾切除术者应用脾B细胞淋巴瘤研究组的最低诊断标准进行诊断。结果表明,5例(26.3%)患者白细胞≥10.0×109/L、6例(31.6%)<4.0×109/L;14例(73.7%)血红蛋白<120 g/L;11例(57.9%)血小板<100.0×109/L;14例(73.8%)患者具有血细胞减少,其中一系血细胞减少6例(31.6%),两系血细胞减少4例(21.1%),三系血细胞减少4例(21.1%);19例均有骨髓累及;10例(52.6%)检出带绒毛的淋巴细胞;淋巴瘤细胞在骨髓内呈现包括窦内浸润的多种分布特点;单克隆免疫球蛋白升高者占56.3%(9/16);存在自身免疫现象者占80%(12/15);4例进行单纯脾切除术,血象均无改善;1例接受脾切除术联合化疗获得部分缓解;11例接受含有利妥昔单抗的治疗,总体反应率100.0%,其中9例(81.8%)获得完全缓解。结论:伴有血细胞计数异常的SMZL以血细胞减少、骨髓高累及率和自身免疫现象为多见,含利妥昔单抗的治疗具有较好的疗效。The aim of this study was to investigate the clinical and laboratorial characteristics of splenic marginal zone lymphoma （SMZL） with an abnormal complete blood count（CBC）. Data of 19 newly diagnosed SMZL patients with abnormal CBC were analyzed retrospectively. Seven patients were diagnosed by using splenic histology, 12 patients who did not undergo splenectomy were diagnosed on the basis of typical clinical presentation and cytologic, immunophenotypic and histologic characteristics of peripheral blood and bone marrow, according to SBLG guidelines. The results showed that leukocytosis（ ≥10.0 × 10^9/L） was seen in 5 cases（26.3% ） ; leukocytopenia（ 〈4.0 × 10^9/L） was found in 6 cases（31.6% ）, hemoglobin concentration less than 120 g/L was found in 14 cases（73.7% ） and thrombocytopenia was found in 11 （57.9%） patients. Fourteen（73.7% ） patients had cytopenia in one or more lineage. As a specific morphologic character, villous lymphocytes were found in 10 （52.6%） patients. Similar immunophynotype was determined by histology in both bone marrow and spleen. Various histological infiltration patterns including intrasinusoidal pattern were found in bone marrow. Nine out of 16 （56.3%） patients displayed an increase of serum monoclonal immunoglobin. Autoimmnne phenomena was found in 12 out of 15 （ 80.0% ） patients. Splenectomy, as the only treatment could not achieve a ≥50% improvement of CBC in 4 patients, and then was judged as no response. Splenectomy followed by chemotherapy achieved partial response（PR） in 1 patient. Overall response rate of the therapeutic strategies with Ritux- imab was 100.0% （11/11）. Furthermore, complete response was achieved in 9 out of 1l （81.8%） patients. It is concluded that SMZL with abnormal CBC has a higher incidence of cytopenia, bone marrow involvement and autoimmune phenomena. Therapeutic strategies consisting of Rituximab show a better efficacy.

关 键 词：脾边缘区淋巴瘤 脾脏 外周血 骨髓 血象异常 

分 类 号：R733.1[医药卫生—肿瘤]