系统性间变性大细胞淋巴瘤骨髓累及的临床病理学研究

Clinicopathological observation of bone marrow involvement of systemic anaplastic large-cell lymphoma

目的探讨系统性间变性大细胞淋巴瘤（S-ALCL）骨髓累及的临床病理学特点、免疫学表型及临床生物学行为。方法回顾性分析34例S-ALCL病例资料，进行骨髓活检（19例）或涂片（15例）。其中ALK（＋）24例，ALK（-）10例。HE染色、免疫组织化学染色观察病理形态及免疫表型，原位杂交法检测EB病毒。结果6例（17．6％）S—ALCL存在骨髓累及，均经骨髓活检标本确定，15例患者骨髓涂片中均未见肿瘤累及。ALK（＋）ALCL和ALK（-）ALCL骨髓累及的发生率分别为16．7％（4／24）和20．0％（2／10），差异无统计学意义（P=0．3555）。与无骨髓累及病例比较，骨髓累及病例的年龄、性别分布差异无统计学意义（P值分别为0．8089和0．3085）。骨髓累及者肿瘤细胞以间质性分布为主[83．3％（5／6）]。生存分析统计提示伴有骨髓累及的患者预后明显差于无骨髓累及者（P=0．0407）。结论S-ALCL骨髓累及发生率低，与患者的发病年龄、性别及ALK蛋白的表达无相关性。伴有骨髓累及的S—ALCL患者临床预后差，骨髓活检在判断S—ALCL预后中有重要意义。

Objective To investigate the clinicopathological features, immunophenotyping and clinical biological behavior of bone marrow （BM） involvement of systemic anaplastic large-cell lymphoma （S-ALCL）. Methods 34 S-ALCL including 24 ALK（＋） and 10 ALK（-） cases available with the formalin-fixed, paraffin embedded （FFPE） tissue blocks of BM biopsy （n=19） or BM smear sections （n=lS） were included in this study. BM samples were sent to both morphologic evaluation using H＆E （Hematoxylin ＆ Eosin）-stained sections and immunophenotypic detection by immunohistochemistry （IHC）. EBV status was determined by visualization of EBERs in tumor cells using in situ hybridization （ISH）. Results BM involvement was seen in 17.6 % （6/34） S-ALCL patients which were confirmed by BM biopsy. No significant difference in the incidence of BM involvement was observed between ALK（＋） [16.7 % （4/24）] and ALK（-） [20.0 % （2/10） S-ALCL （P =0.3555）. Age and gender were not associated with the presence or the absence of BM involvement by S-ALCL （P = 0.8089 and 0.3085）, tumor cells of patients with BM involvement were interstitial distribution. S-ALCL patients with BM involvement have a poor prognosis as compared to those without BM involvement （P =0.0407）. Conclusion BM involvement was not frequently seen in S-ALCL. The occurrence of BM involvement by S-ALCL was not associated with age, gender or the expression of ALK protein. BM involvement is an adverse prognostic factor in S-ALCL, BM biopsy is useful to predict the prognosis of S-ALCL.