淋巴浆细胞性淋巴瘤的临床病理特点分析

Clinicopathologic features of lymphoplasmacytic lymphoma

目的探讨淋巴浆细胞性淋巴瘤（LPL）的临床及病理学特点及其鉴别诊断和与Waldenstrom巨球蛋白血症的关系。方法24例骨髓活检标本，6例同时行淋巴结活检，行石蜡包埋切片、HE染色形态观察及免疫组织化学EliVision法检测分析。结果男17例，女7例（男：女=2．4：1）。中位年龄59．5岁（42～75岁）。临床表现以乏力最多见，为83．3％（20／24）。高黏滞血症20．8％（5／24），B症状8．3％（2／24），浅表淋巴结肿大41．7％（10／24）。贫血79．2％（19／24），白细胞增高8．3％（2／24），血小板减少37．5％（9／24）。血清免疫固定电泳显示23例（95．8％）出现单克隆性免疫球蛋白轻链条带，IgM型20例、IgG型2例、IgA型1例。22例骨髓活检和2例淋巴结活检均经病理组织形态和免疫组织化学诊断为LPL，骨髓及淋巴结瘤细胞由小淋巴细胞、浆细胞样淋巴细胞及浆细胞组成；侵犯骨髓的方式多为弥漫型（63．6％，14／22），结节型及问质型少见分别为22．7％（5／22）及13．6％（3／22）。淋巴结瘤细胞呈弥漫性分布。瘤细胞表达Pax5、CD20、CD38、CD138，不表达CD5、CD10、CD23、细胞周期蛋白D1、CD3、CD7、髓过氧化物酶。结论LPL具有明确的临床及病理学特点，诊断应主要结合组织形态和免疫表型与慢性淋巴细胞白血病／小细胞淋巴瘤、脾脏边缘区淋巴瘤及滤泡性淋巴瘤等小淋巴细胞肿瘤鉴别。Waldenstrom巨球蛋白血症的本质为LPL。

Objective To explore the clinicopathologic features of lymphoplasmacytic lymphomas （LPL）. Methods Routine histological examination was performed on hematoxylin-eosin stained sections of 24 bone marrow biopsies and available 6 concurrent lymph node specimens. Immunohistoehemistry study was performed using EliVision methods. Results Among 24 cases, the male-to-female ratio was 2. 4：1 and the median age was 59. 5 years （ 42-75 ）. The most common symptom was weakness （ 83.3%, 20/24 ）. Hyperviscosity and ＂ B＂ symptoms occurred in 20. 8% （5/24） and 8.3% （2/24） respectively. 41.7% （10/24） patients presented with lymphadenopathy. Anemia, leukoeytosis and thrombocytopenia were seen in 79. 2% （19/24）, 8. 3% （2/24） and 37. 5% （9/24） respectively. Monoclonal Ig light chain expression was detected by serum immunofixation electrophoresis in 23 cases（95.8% ） ,including IgM （20 cases） , IgG （2 eases） and IgA （1 ease）. Basing on the histology and immunohistoehemistry findings, the diagnosis was made in 22 bone marrow and 2 lymph node biopsies, respectively. Histologically, the bone marrow and lymph node specimens composed of small lymphocytes,plasmaeytoid lymphoeytes and plasma cells. The most frequent pattern of bone marrow involvement was diffuse in appearanee （63.6% , 14/22） ,while nodular and interstitial patterns were less common （22. 7% , 5/22 and 13.6% , 3/22, respectively） . Lymph node involvement was also to be diffuse in pattern. The proliferative eells expressed Pax5, CD20, CD38 and CD138, but were negative for CD5,CD10, CD23, CyelinD1, CD3, CD7 and MPO. Conclusions LPL has distinct elinieopathologieal features. Histological and immunohistochemistry findings are important for its differential diagnosis with chronic lymphocytic leukemia/small lymphocytie lymphoma, splenic marginal zone lymphoma and follicular lymphoma. Waldenstrom macroglobulinemia is LPL.