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目的探讨髓外浆细胞瘤(Extramedullary plasmacytoma,EMP)的诊断和鉴别诊断。方法对16例EMP的组织形态学及免疫表型特征进行研究,并与3例髓外多发性骨髓瘤(Extr-amedullary multiple myeloma,EMM)、3例髓内多发性骨髓瘤(medullary multiple myeloma,MM)及10例浆细胞反应性增生作比较。结果16例EMP,Ⅰ级2例,Ⅱ级12例(富浆型9例,少浆型3例),Ⅲ级2例。3例EMM为Ⅱ级少浆及富浆型各1例,Ⅲ级1例;3例MM均为Ⅱ级(富浆型2例,少浆型1例)。10例浆细胞反应性增生均为成熟浆细胞,常混有淋巴细胞和组织细胞。免疫组化显示16例EMP均呈轻链限制性,CD20、CD56及CyclinD1均阴性;CD138 62.5%(10/16)阳性,Bcl-237.5%(6/16)阳性,但均强度较弱;CD79a 71.4%(10/14)阳性,LCA 42.8%(6/14)阳性,EMA28.5%(2/7)阳性。3例EMM和MM均呈轻链限制性,CD138均阳性。CD56阳性4例(含3例EMM),Bcl-2阳性5例(含3例EMM),均强阳性。10例浆细胞反应性增生均无轻链限制性。结论Ⅰ级EMP应与浆细胞反应性增生相鉴别;Ⅱ级者应与小B细胞淋巴瘤、粒细胞肉瘤及浆细胞样肌上皮瘤相鉴别;Ⅲ级者应与大细胞性淋巴瘤、恶性黑色素瘤、低分化癌、肌源性肉瘤及肾外横纹肌样瘤相鉴别。CD56和Bcl-2可能是鉴别EMP和EMM的有用标记。
Objective To investigate the diagnosis and differential diagnosis of extramedullary plasmacytoma (EMP). Methods The histomorphological and immunophenotypic features of 16 cases of EMP were studied, and compared with 3 cases of extramedullary multiple myeloma (EMM) and 3 cases of multiple myeloma (medullary multiple myeloma). MM) and 10 cases of plasma cell reactive hyperplasia were compared. Results There were 16 cases of EMP, 2 cases of grade I, 12 cases of grade II (9 cases of rich plasma, 3 cases of less pulp), and 2 cases of grade III. 3 cases of EMM were type II less pulp and pulp-rich type 1 case, and grade III 1 case; 3 cases MM were grade II (rich slurry type 2 cases, less pulp type 1 case). All 10 cases of plasma cell reactive hyperplasia were mature plasma cells, often mixed with lymphocytes and histiocytes. Immunohistochemistry showed that all 16 EMPs were light chain-restricted, and CD20, CD56, and CyclinD1 were all negative; CD138 was 62.5% (10/16) positive, and Bcl-237.5% (6/16) was positive, but the average intensity was weak; CD79a 71.4% (10/14) were positive, LCA was 42.8% (6/14) positive, and EMA was 28.5% (2/7) positive. All three cases of EMM and MM were light chain-restricted and all of CD138 were positive. CD56 positive in 4 cases (including 3 cases of EMM), Bcl-2 positive in 5 cases (including 3 cases of EMM), are strongly positive. None of the 10 plasma cell reactive hyperplasias had light chain restriction. Conclusions Grade I EMP should be differentiated from plasma cell reactive hyperplasia; grade II should be differentiated from small B-cell lymphoma, granulocyte sarcoma and plasmacytoid myoepithelial neoplasia; grade III should be associated with large cell lymphoma, malignant Melanoma, poorly differentiated carcinoma, myogenic sarcoma, and extrarenal rhabdoid tumors were identified. CD56 and Bcl-2 may be useful markers for identifying EMPs and EMMs.