Ree H J, Kadin M E, Kikuchi M, Ko Y H, Go J H, Suzumiya J, Kim D S
Samsung Medical Center, Seoul, Korea.
Am J Surg Pathol. 1998 Jun;22(6):643-55. doi: 10.1097/00000478-199806000-00001.
Angioimmunoblastic T-cell lymphoma (or angioimmunoblastic lymphadenopathy with dysgammaglobulinemia [AILD]) was originally considered to be an abnormal immune reaction in which reactive follicles with germinal centers (GCs) are usually absent. When hyperplastic GCs are present along with an angioimmunoblastic reaction, the lesion has been interpreted as a benign hyperimmune reaction. We report seven patients with angioimmunoblastic T-cell lymphoma (AITL) who initially had hyperplastic GCs, shown to be malignant lymphoma by further studies and clinical follow-up. Clonal T-cell populations were observed in all specimens evaluated, and sequential biopsies showed histologic progression to typical AITL in two patients. Clinical presentation was characterized by generalized lymphadenopathy of acute onset, constitutional symptoms, hepatosplenomegaly, skin rash, and polyclonal hypergammaglobulinemia in five patients; regional adenopathy preceded generalized adenopathy in two patients. Five patients had rapid progression of disease, and three patients whose treatment was delayed due to inadequate evidence to diagnose lymphoma died of infection. The initial biopsy findings of each patient were similar and showed angioimmunoblastic proliferation, hyperplastic GCs with ill-defined borders, and interfollicular tingible-body macrophages. These GCs differed from occasional residual follicles of typical AITL in that the GCs were enlarged and hyperplasia of follicular dendritic cells was not seen. Diagnostic clear cells were not observed. Apoptotic bodies were markedly increased and bcl-2+ lymphocytes were sparse compared with typical AITL. Results of in situ hybridization for Epstein-Barr virus were positive in each case. We conclude that hyperplastic germinal centers with ill-defined borders and frequent interfollicular tingible-body macrophages occur in a histologic variant of AITL that is necessary to recognize for early diagnosis and treatment.
血管免疫母细胞性T细胞淋巴瘤（或伴γ球蛋白异常血症的血管免疫母细胞性淋巴结病[AILD]）最初被认为是一种异常免疫反应，通常不存在带有生发中心（GCs）的反应性滤泡。当增生的GCs与血管免疫母细胞反应同时存在时，该病变被解释为良性高免疫反应。我们报告了7例血管免疫母细胞性T细胞淋巴瘤（AITL）患者，他们最初有增生的GCs，经进一步研究和临床随访显示为恶性淋巴瘤。在所有评估的标本中均观察到克隆性T细胞群体，连续活检显示2例患者组织学进展为典型的AITL。临床表现的特征为5例患者急性起病的全身淋巴结肿大、全身症状、肝脾肿大、皮疹和多克隆高球蛋白血症；2例患者局部淋巴结病先于全身淋巴结病出现。5例患者疾病进展迅速，3例因诊断淋巴瘤证据不足而治疗延迟的患者死于感染。每位患者的初始活检结果相似，显示血管免疫母细胞增生、边界不清的增生性GCs以及滤泡间嗜染体巨噬细胞。这些GCs与典型AITL偶尔残留的滤泡不同，在于GCs增大且未见滤泡树突状细胞增生。未观察到诊断性透明细胞。与典型AITL相比，凋亡小体明显增多，bcl-2+淋巴细胞稀少。每例患者的爱泼斯坦-巴尔病毒原位杂交结果均为阳性。我们得出结论，边界不清的增生性生发中心和频繁的滤泡间嗜染体巨噬细胞出现在AITL组织学变异型中，这对于早期诊断和治疗是必须识别的。
