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多中心性Castleman病的骨髓表现

Bone marrow manifestations in multicentric Castleman disease.

作者信息

Ibrahim Hazem A H, Balachandran Kirsty, Bower Mark, Naresh Kikkeri N

机构信息

Department of histopathology, Hammersmith Hospital, Imperial College London, London, UK.

Department of histopathology, Faculty of Medicine, Mansoura University, Egypt.

出版信息

Br J Haematol. 2016 Mar;172(6):923-9. doi: 10.1111/bjh.13919. Epub 2016 Jan 28.

DOI:10.1111/bjh.13919
PMID:26817834
Abstract

This study aimed to document the morphological and immunophenotypic features, and describe the diagnostic features of bone marrow (BM) involvement in human herpes virus 8 Multicentric Castleman disease (HHV8-MCD). BM trephine biopsy (BMTB) specimens from 28 patients were revisited. Samples were evaluated for expression of CD3, CD20, CD138, CD68R, glycophorin C, CD42b, HHV8-latency-associated nuclear antigen (LANA1), Epstein-Barr virus-encoded small RNA and light chains. Presence of significant numbers of HHV8-LANA1(+) lymphoid/plasmacytic cells, noted in 10/28 cases, was indicative of BM involvement and was associated with low CD4 and CD8 counts in peripheral blood. The characteristic morphological appearance of MCD seen in lymph nodes is a rare finding in BMTB. 4/5 cases with lymphoid aggregates were involved by MCD, whereas 6/23 cases without lymphoid aggregates were involved by MCD (P = 0·023). 9/18 cases with hypercellular marrow were involved by MCD, whilst only 1/8 cases with normo/hypocellular marrow showed involvement by MCD (P = 0·070). While 9/21 cases with increased marrow reticulin were involved by MCD, none of the cases with no increase in reticulin were involved by MCD (P = 0·080). Reactive plasmacytosis is a frequent finding. We conclude that bone marrow is involved in a significant proportion of patients with MCD (36%), and involvement can be identified by HHV8-LANA1 immunohistochemistry.

摘要

本研究旨在记录形态学和免疫表型特征,并描述人类疱疹病毒8型多中心Castleman病(HHV8-MCD)骨髓(BM)受累的诊断特征。对28例患者的骨髓活检(BMTB)标本进行了复查。评估样本中CD3、CD20、CD138、CD68R、血型糖蛋白C、CD42b、HHV8潜伏相关核抗原(LANA1)、爱泼斯坦-巴尔病毒编码的小RNA和轻链的表达情况。在10/28例病例中发现大量HHV8-LANA1(+)淋巴样/浆细胞,这表明存在骨髓受累,并且与外周血中CD4和CD8计数低有关。在骨髓活检中很少见到在淋巴结中出现的MCD特征性形态表现。5例有淋巴样聚集的病例中有4例存在MCD累及,而23例无淋巴样聚集的病例中有6例存在MCD累及(P = 0·023)。18例骨髓细胞增多的病例中有9例存在MCD累及,而8例骨髓细胞正常/减少的病例中只有1例存在MCD累及(P = 0·070)。21例骨髓网硬蛋白增加的病例中有9例存在MCD累及,而网硬蛋白未增加的病例中无一例存在MCD累及(P = 0·080)。反应性浆细胞增多是常见表现。我们得出结论,相当一部分MCD患者(36%)存在骨髓受累,通过HHV8-LANA1免疫组化可识别这种受累情况。

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