Abi-Rafeh Jad, Beamish Ian V, Haegert David G, Cournoyer Denis, Michel René P
Faculty of Medicine.
Department of Pathology.
Medicine (Baltimore). 2020 May;99(19):e20106. doi: 10.1097/MD.0000000000020106.
Richter syndrome (RS) defines the transformation of chronic lymphocytic leukemia (CLL) into a more aggressive lymphoma. Although the term RS is most often reserved for transformation of CLL into diffuse large B-cell lymphoma (DLBCL), and less frequently Hodgkin lymphoma , the list of cases with more variable presentations in the literature is growing.
A 71-year-old Caucasian man initially consulted an otolaryngologist for a 1-year history of nasal congestion.
The asynchronous occurrence of 2 rare angiocentric Epstein-Barr virus (EBV)-related lymphoproliferative disorders in a patient with CLL, specifically clonally related lymphomatoid granulomatosis (LYG), and an extranodal NK/T-cell lymphoma, nasal type, are described herein.
Radiation therapy and a regimen of cis-platinum were administered for the NK/T cell lymphoma, and ibrutinib for LYG.
The patient remains in complete clinical remission 8 years after the diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma and recurrent extranodal NK/T cell lymphoma, and 2 years after the diagnosis of clonally related LYG.
Although the precise pathogenesis of RS remains incompletely understood, various molecular alterations, in particular long-term immunosuppression, may lead to RS, similar to the causal link existing between non-Hodgkin lymphomas and HIV infection, and post-transplantation lymphoproliferative disorders. EBV infection is linked to the pathogenesis of several types of lymphomas and found in a subset of patients with RS; immunosuppression, in the context of CLL or other pathological conditions or pharmacological agents, can disrupt the fine balance between virus and the host immune system, and result in EBV-driven lymphoproliferations of B-, T-, or NK-cell origin. The findings of our literature review thus suggest that such non-diffuse large B-cell lymphoma , non-Hodgkin lymphoma CLL transformations, may be considered as rare variants of RS.
里氏综合征（RS）定义为慢性淋巴细胞白血病（CLL）转化为侵袭性更强的淋巴瘤。尽管RS一词最常指CLL转化为弥漫性大B细胞淋巴瘤（DLBCL），较少指霍奇金淋巴瘤，但文献中呈现出更多样化表现的病例列表正在增加。
一名71岁的白人男性因鼻塞1年，最初咨询了耳鼻喉科医生。
本文描述了一名CLL患者同时发生2种罕见的血管中心性爱泼斯坦-巴尔病毒（EBV）相关淋巴增殖性疾病，具体为克隆相关的淋巴瘤样肉芽肿病（LYG）和鼻型结外NK/T细胞淋巴瘤。
对NK/T细胞淋巴瘤进行了放射治疗和顺铂方案治疗，对LYG进行了伊布替尼治疗。
在慢性淋巴细胞白血病/小淋巴细胞淋巴瘤和复发性结外NK/T细胞淋巴瘤诊断8年后，以及克隆相关的LYG诊断2年后，患者仍处于完全临床缓解状态。
尽管RS的确切发病机制仍未完全了解，但各种分子改变，特别是长期免疫抑制，可能导致RS，这类似于非霍奇金淋巴瘤与HIV感染以及移植后淋巴增殖性疾病之间存在的因果联系。EBV感染与几种淋巴瘤的发病机制有关，并且在一部分RS患者中发现；在CLL或其他病理状况或药物作用的背景下，免疫抑制会破坏病毒与宿主免疫系统之间的精细平衡，并导致B细胞、T细胞或NK细胞来源的EBV驱动的淋巴增殖。因此，我们的文献综述结果表明，这种非弥漫性大B细胞淋巴瘤、非霍奇金淋巴瘤CLL转化，可能被视为RS的罕见变体。
