FLORID PROGRESSIVE TRANSFORMATION OF GERMINAL-CENTERS - A SYNDROME AFFECTING YOUNG MEN, WITHOUT EARLY PROGRESSION TO NODULAR LYMPHOCYTE PREDOMINANCE HODGKINS-DISEASE
FLORID PROGRESSIVE TRANSFORMATION OF GERMINAL-CENTERS - A SYNDROME AFFECTING YOUNG MEN, WITHOUT EARLY PROGRESSION TO NODULAR LYMPHOCYTE PREDOMINANCE HODGKINS-DISEASE
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DOI：
10.1097/00000478-199203000-00005
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发表时间：
1992-03-01
影响因子：
5.6
通讯作者：
HARRIS, NL
中科院分区：
文献类型：
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作者：
FERRY, JA;ZUKERBERG, LR;HARRIS, NL
生发中心进行性转化(PTGC)发生在反应性淋巴结局灶性病变，在以结节淋巴细胞为主的何杰金氏病(NLPHD)患者中发生率增加。有研究表明，淋巴结活检样本显示为PTGC的患者患NLPHD的风险增加，何杰金氏病可能从PTGC演变而来。我们报告5名年轻男性(年龄14-24岁，平均18岁)，他们有明显的淋巴结病和鲜艳的PTGC，仔细的检查和随访显示他们没有进展到霍奇金氏病。三名患者发展为多个结节组的腺病，两名患者有局限性的腺病。切除颈部(2例)、腹股沟(2例)和腋窝(1例)3~4 cm的淋巴结。每个标本的渐进性转化生发中心(PTGCs)数量为10~123个(平均67个)，单个切片包含9~29个PTGCs(平均19个)。3例结节结构明显扭曲，提示NLPHD，但Reed-Sternberg细胞缺失。所有患者均可获得随访(均未治疗)：3名患者在确诊后1年、4个月至10年内出现持续性腺病。两名患者(确诊后2年和3年)的重复活检结果显示，PTGC颜色鲜艳，没有霍奇金氏病的证据。其中一名患者在就诊8年后进行了一次随后的活检；结果显示只有罕见的PTGCs。第四组和第五组患者出现孤立性腺病，两年和五年后均无复发。这些病例提示青春期男孩和年轻男子的淋巴组织增生症伴花状PTGC。虽然腺病可以持续，但没有进展到霍奇金氏病。认识这一综合征对于避免LPHD的误诊是很重要的。有必要对这些患者进行密切随访，以评估这种疾病与NLPHD的关系。
Progressive transformation of germinal centers (PTGC) occurs focally in reactive lymph nodes, and has been reported with increased frequency in patients with nodular lymphocyte predominance Hodgkin's disease (NLPHD). It has been suggested that patients with lymph node biopsy samples showing PTGC are at increased risk for the development of NLPHD, and that Hodgkin's disease may evolve from PTGC. We report five young men (ages 14-24 years, mean 18) with prominent lymphadenopathy and florid PTGC, in whom careful examination and follow-up showed no progression to Hodgkin's disease. Three patients developed adenopathy that involved several node groups and two had localized adenopathy. Cervical (2), inguinal (2) and axillary (1) nodes ranging from 3 to 4 cm were excised. The number of progressively transformed germinal centers (PTGCs) ranged from 10 to 123 per specimen (mean 67); single sections contained nine to 29 PTGCs (mean 19). In three cases the nodal architecture was significantly distorted, suggestive of NLPHD, but Reed-Sternberg cells were absent. Follow-up is available for all patients (all untreated): three patients had persistent adenopathy 1 year 4 months to 10 years after diagnosis. Results of repeat biopsy in two patients (2 and 3 years after diagnosis) showed florid PTGC with no evidence of Hodgkin's disease. One of these patients had one subsequent biopsy 8 years after presentation; results showed only rare PTGCs. The fourth and fifth patients, who had presented with isolated adenopathy, were free of recurrent adenopathy at 2 and 5 years. These cases suggest a syndrome of lymphoid hyperplasia with florid PTGC in adolescent boys and young men. Although adenopathy can persist, there has been no progression to Hodgkin's disease. Recognition of this syndrome is important to avoid overdiagnosis of LPHD. Close follow-up of these patients will be necessary to evaluate the relationship of this disorder to NLPHD.