Histologic transformation of t(11;18)-positive MALT lymphoma presented with aberrant T-cell marker expression
Histologic transformation of t(11;18)-positive MALT lymphoma presented with aberrant T-cell marker expression
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DOI：
10.1007/s12185-019-02810-y
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发表时间：
2020-05-01
影响因子：
2.1
通讯作者：
Takaori-Kondo, Akifumi
中科院分区：
文献类型：
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作者：
Tamura, Naoki;Maeda, Hirona;Takaori-Kondo, Akifumi
粘膜相关淋巴组织（MALT）淋巴瘤伴t(11;18)(q21;q21)，导致API2-MALT1融合转录物，据报道很少转化为侵袭性淋巴瘤。在这里，我们报告了一位t（11;18）阳性MALT淋巴瘤患者在20年的病史后经历组织学转变的临床过程。患者突然出现巨大的盆腔内肿块和腹水，乳酸脱氢酶迅速升高。腹水细胞学检查发现大的异常细胞，流式细胞术分析发现，这些细胞细胞质CD3、CD4和CD38阳性，CD7部分阳性，CD19和CD20阴性。抗原受体基因重排分析和免疫球蛋白轻链原位杂交证实肿瘤细胞为b细胞系。染色体分析显示复杂的核型与克隆内变异，除了t(11;18), t（8;14）和杂合丢失的TP53。尽管与原始MALT淋巴瘤相比，组织学和表型特征发生了明显改变，但t（11;18）的存在使我们诊断为MALT淋巴瘤的组织学转化。虽然t（11;18）阳性MALT淋巴瘤向侵袭性淋巴瘤的转变极为罕见，但也可能发生，可能伴有额外的遗传异常，如cMYC重排和/或TP53的缺失。
Mucosa-associated lymphoid tissue (MALT) lymphoma with t(11;18)(q21;q21), resulting in an API2-MALT1 fusion transcript, is reported to rarely transform into aggressive lymphoma. Here, we report the clinical course of a patient who experienced histologic transformation after 20 years' disease history of t(11;18)-positive MALT lymphoma. The patient suddenly developed a large intrapelvic mass and ascites with a rapid increase in lactate dehydrogenase. Cytology of the ascites detected large abnormal cells, and flow cytometric analysis revealed that the cells were positive for cytoplasmic CD3, CD4, and CD38, and partially positive for CD7, but negative for CD19 and CD20. Antigen receptor gene rearrangement analysis and in situ hybridization of the immunoglobulin light chains confirmed that the tumor cells were of B-cell lineage. Chromosomal analysis showed complex karyotypes with intraclonal variation, and in addition to t(11;18), t(8;14) and heterozygous loss of the TP53 were demonstrated. Although histological and phenotypic features were significantly altered from the original MALT lymphoma, the presence of t(11;18) led us to the diagnosis of histologic transformation of MALT lymphoma. Although transformation of t(11;18)-positive MALT lymphoma into aggressive lymphoma is extremely rare, it may occur, probably with additional genetic abnormalities such as cMYC rearrangement and/or the loss of TP53.