Peripheral T-cell lymphoma of Lennert type complicated by monoclonal proliferation of large B-cells

Peripheral T-cell lymphoma of Lennert type complicated by monoclonal proliferation of large B-cells
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DOI:
10.1016/j.prp.2009.04.009
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发表时间:
2010-01-01
影响因子:
2.8
通讯作者:
Aozasa, Katsuyuki
Aozasa, Katsuyuki
中科院分区:
医学4区
文献类型:
--
作者:
Chihara, Takeshi;Wada, Naoki;Aozasa, Katsuyuki

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一位69岁男性,因右侧腹股沟区淋巴结肿大就诊。进行活检(LN 1),诊断为外周T细胞淋巴瘤。在联合化疗后约51个月,病灶完全缓解,但38例患者出现肿胀性丘疹、全身淋巴结病和发热。对皮肤(S1)和淋巴结(LN 2)进行活检。红斑性丘疹曾经自发消失,但再次出现并进行活检(S2)。LN 1表现出典型的Lennert淋巴瘤的组织学和免疫组化特征,即,CD 3+、CD 4+、CD 8-免疫表型的小到大淋巴细胞的弥漫性增殖,伴有大量上皮样组织细胞簇。在LN 2中,具有CD 3+、CD 4+、CD 8-的大细胞数量减少,而可观察到大量CD 20+大细胞。克隆性分析揭示了在LN 1和LN 2中持续存在相同的T细胞克隆。免疫球蛋白重链(IgH)基因的克隆带在LN 2中检测到,但在LN 1中没有。S1、S2真皮上层CD 20-、CD 3+、CD 4+、CD 8-淋巴样细胞由小到大弥漫性增生,具有明显的向表皮性。克隆性分析显示存在与LN 1和LN 2相同的T细胞克隆,而没有B细胞克隆,表明PTCL复发。EB病毒(EBV)基因组原位杂交(ISH)结果显示,大B淋巴细胞核内的阳性信号仅出现在LN 2中。(c)2009年Elsevier GmbH。All rights reserved.
A 69-year-old man presented with lymph node swelling in the right inguinal region. A biopsy was made (LN1) and diagnosed as peripheral T-cell lymphoma. The lesion remitted completely over a period of about 51 months after combination chemotherapy, but erythematous papules, systemic lymphadenopathy, and fever of 38 appeared. Skin (S1) and lymph nodes (LN2) were biopsied. Erythematous papules once disappeared spontaneously, but appeared again and were biopsied (S2). LN1 displayed the typical histologic and immunohistochemical features of Lennert lymphoma, i.e., diffuse proliferation of small to large lymphoid cells of CD3+, CD4+, CD8- immunophenotype accompanied by numerous clusters of epithelioid histiocytes. In LN2, the large cells with CD3+, CD4+, CD8- decreased in number, while numerous CD20+ large cells were discernible. Clonality analysis revealed the persistent presence of an identical T-cell clone in LN1 and LN2. Clonal bands of immunoglobulin heavy (IgH) chain gene were detected in LN2 but not in LN1. Si and S2 showed diffuse proliferation of small to large lymphoid cells of CD20-, CD3+, CD4+, CD8- in the upper dermis, with obvious epidermotropism. Clonality analysis revealed the presence of a T-cell clone identical to LN1 and LN2 with no B-cell clone, indicating the recurrence of PTCL. In situ hybridization (ISH) for Epstein-Barr virus (EBV) genome revealed that positive signals in the nucleus of large B-lymphoid cells appeared only in LN2.Taken together, EBV-positive large B-cell lymphoma appeared transiently in the course of "Lennert lymphoma". (c) 2009 Elsevier GmbH. All rights reserved.