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[小儿伯基特淋巴瘤累及骨髓的病理诊断]

[Pathological diagnosis of pediatric Burkitt lymphoma involving bone marrow].

作者信息

Sun Qi, Chen Zhenping, Liu Enbin, Li Zhanqi, Yang Qingying, Sun Fujun, Ma Yue, Zhang Hongju, Zhang Peihong, Ru Kun

机构信息

Department of Pathology, Hospital of Blood Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin 300020, China.

E-mail:

出版信息

Zhonghua Bing Li Xue Za Zhi. 2015 Feb;44(2):95-9.

Abstract

OBJECTIVE

To investigate pathologic and differential diagnostic features of pediatric Burkitt lymphoma (BL).

METHODS

A total of 20 cases of pediatric BL were retrospectively reviewed for their clinical and pathologic profiles. Bone marrow aspiration specimens were available in all cases and bone marrow biopsies were available for immunohistochemical study in 18 cases. Flow cytometry study was available in 16 cases. MYC translocation by FISH method was performed in 11 cases.

RESULTS

Atypical lymphocytes with cytoplasmic vacuoles were found in bone marrow smears in all 20 cases and peripheral blood films in all 19 available cases. The bone marrow biopsies showed infiltration by uniform medium-sized atypical lymphocytes with multiple small nucleoli but without the starry-sky pattern in all 18 cases. Immunohistochemistry showed the following results in all 18 cases: positive for CD20, PAX-5, CD10, CD34 and TdT, but negative for bcl-2 and CD3 with Ki-67 > 95%.Flow cytometry showed CD19+CD20+CD10+FMC7+CD22+TdT-CD3- in 16 cases, including κ+ in 8 cases, λ+ in 7 cases, and κ-λ- in 1 case. MYC gene rearrangement by FISH was observed in 10 of the 11 cases.

CONCLUSIONS

The histopathology of BL is distinct, including atypical lymphocytes with cytoplasmic vacuoles in bone marrow aspirate, lack of starry-sky patternin bone marrow biopsy. Generally, the diagnosis should be made with a combined immunophenotype and FISH approach. Pediatric BL must be distinguished from DLBCL and B-cell lymphoma, unclassifiable, which has intermediate features between DLBCL and Burkitt lymphoma.

摘要

目的

探讨儿童伯基特淋巴瘤(BL)的病理及鉴别诊断特征。

方法

回顾性分析20例儿童BL的临床和病理资料。所有病例均有骨髓穿刺标本,18例有骨髓活检标本用于免疫组织化学研究,16例可行流式细胞术检测。11例采用荧光原位杂交(FISH)法检测MYC基因易位。

结果

20例骨髓涂片及19例可获得的外周血涂片均发现有胞质空泡的非典型淋巴细胞。18例骨髓活检均显示为中等大小的非典型淋巴细胞浸润,有多个小核仁,但无满天星现象。免疫组织化学检测18例结果如下:CD20、PAX - 5、CD10、CD34和TdT阳性,bcl - 2和CD3阴性,Ki - 67>95%。流式细胞术检测16例显示为CD19+CD20+CD10+FMC7+CD22+TdT - CD3 -,其中8例κ阳性,7例λ阳性,1例κ - λ - 。11例中10例通过FISH检测到MYC基因重排。

结论

BL的组织病理学特征明显,包括骨髓穿刺涂片中出现有胞质空泡的非典型淋巴细胞,骨髓活检中无满天星现象。一般来说,诊断应结合免疫表型和FISH方法。儿童BL必须与弥漫性大B细胞淋巴瘤(DLBCL)及具有DLBCL和伯基特淋巴瘤中间特征的不可分类B细胞淋巴瘤相鉴别。

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