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弥漫性大 B 细胞淋巴瘤和外周 T 细胞淋巴瘤在 11 年前诊断为多中心 Castleman 病后出现淋巴细胞耗竭的经典霍奇金淋巴瘤。

Discordant lymphocyte-depleted classical Hodgkin's and peripheral T-cell lymphoma arising in a patient 11 years after diagnosis of multicentric Castleman's disease.

机构信息

Department of Laboratory Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.

出版信息

Int J Hematol. 2013 Jul;98(1):114-21. doi: 10.1007/s12185-013-1358-0. Epub 2013 Jun 4.

Abstract

Castleman's disease (CD) is thought to be related with an initially benign viral disease with cytokine-driven propagation and malignant transformation. This paper reports the first case of a simultaneous discordant lymphoma consisting of lymphocyte-depleted classical Hodgkin's lymphoma (LDCHL) and peripheral T-cell lymphoma (PTCL) arising in a patient with multicentric CD (MCD). PTCL occurred 4 years after the diagnosis of MCD, and LDCHL was developed 6 years after the treatment of PTCL, sequentially. The following year, the patient presented with a relapse of a simultaneous discordant lymphoma. On excisional cervical LN biopsy, immunohistochemical stain pattern was identical with previously diagnosed LDCHL, which expressed CD30, CD15, PAX5, and Epstein-Barr virus (EBV)-encoded RNA. PTCL was positive for CD3, CD4, CD5, CD10, and CD56, and showed identical TCRB and TCRG gene rearrangements to those detected initially. MCD was thought to be the major contributing factor leading to initial PTCL, while EBV-positive LDCHL is thought to have promoted the development of PTCL, as a persistently abnormal immune microenvironment may induce the recurrence of PTCL. MCD runs a more aggressive course and can progress to Hodgkin's lymphoma (HL), non-Hodgkin's lymphoma (NHL), or combined HL/NHL. Due to its malignant potential, prompt recognition and therapy is critical for these situations, which may be life threatening.

摘要

卡斯尔曼病(CD)被认为与最初的良性病毒性疾病有关,其具有细胞因子驱动的增殖和恶性转化。本文报道了首例同时发生的淋巴瘤,包括淋巴细胞耗竭型经典霍奇金淋巴瘤(LDCHL)和外周 T 细胞淋巴瘤(PTCL),发生在多中心 CD(MCD)患者中。PTCL 发生在 MCD 诊断后 4 年,LDCHL 在 PTCL 治疗后 6 年后发生,顺序发生。次年,患者出现同时发生的淋巴瘤复发。在切除性颈淋巴结活检中,免疫组织化学染色模式与先前诊断的 LDCHL 相同,LDCHL 表达 CD30、CD15、PAX5 和 Epstein-Barr 病毒(EBV)编码的 RNA。PTCL 对 CD3、CD4、CD5、CD10 和 CD56 呈阳性,并且 TCRB 和 TCRG 基因重排在最初检测到的基因重排相同。MCD 被认为是导致初始 PTCL 的主要因素,而 EBV 阳性 LDCHL 被认为促进了 PTCL 的发展,因为持续异常的免疫微环境可能会导致 PTCL 的复发。MCD 病程更具侵袭性,可进展为霍奇金淋巴瘤(HL)、非霍奇金淋巴瘤(NHL)或混合 HL/NHL。由于其恶性潜能,对这些情况的及时识别和治疗至关重要,否则可能会危及生命。

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