Kawano Noriaki, Beppu Kiichiro, Oyama Mayumi, Himeji Daisuke, Yoshida Shuro, Kuriyama Takuro, Ono Nobuyuki, Masuyama Hiroyuki, Yamashita Kiyoshi, Yamaguchi Kenichiro, Shimao Yoshiya, Oshima Koichi, Ueda Yuji, Ueda Akira
Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital.
J Clin Exp Hematop. 2013;53(3):241-5. doi: 10.3960/jslrt.53.241.
Crystal-storing histiocytosis is a rare clinical entity characterized by an increase in the number of abnormal histiocytes accompanied by accumulation of crystallized immunoglobulins. We describe the case of an 80-year-old man who presented with crystal-storing histiocytosis of the lung 13 years after receiving a diagnosis of gastric non-Hodgkin lymphoma (NHL ; clinical stage, Lugano IA). After wedge resection of the left upper lobe, the histological findings showed crystal-storing histiocytosis with CD68(+), some small to medium lymphoid cells with CD79a(+) with κ(+(weekly)) and λ(-), and some plasma cells with CD138(+), and rearrangement of the immunoglobulin heavy chain. Based on the nonrecurrent gastric NHL, small B-cell population, and failure to detect the same clone by polymerase chain reaction analysis, our case was classified as pulmonary localized crystal-storing histiocytosis without underlying lymphoproliferative or plasma cell disorder. The findings of minor B-cell populations harboring a heavy chain rearrangement with slight light-chain restriction (κ > λ) may be related to the pathogenesis of crystallogenesis and crystal-storing histiocytosis. Moreover, surgical treatment may be an effective therapeutic option for solitary crystal-storing histiocytosis.
晶体储存组织细胞增多症是一种罕见的临床病症,其特征为异常组织细胞数量增加,并伴有结晶免疫球蛋白的蓄积。我们报告一例80岁男性病例,该患者在被诊断为胃非霍奇金淋巴瘤(NHL;临床分期,卢加诺IA期)13年后出现肺部晶体储存组织细胞增多症。左上叶楔形切除术后,组织学检查结果显示为晶体储存组织细胞增多症,伴有CD68(+)、一些CD79a(+)且κ(+(每周))和λ(-)的中小淋巴细胞,以及一些CD138(+)的浆细胞,免疫球蛋白重链发生重排。基于非复发性胃NHL、小B细胞群以及聚合酶链反应分析未检测到相同克隆,我们的病例被归类为无潜在淋巴增殖性或浆细胞疾病的肺部局限性晶体储存组织细胞增多症。携带有重链重排且轻链限制轻微(κ>λ)的小B细胞群的发现可能与晶体形成和晶体储存组织细胞增多症的发病机制有关。此外,手术治疗可能是孤立性晶体储存组织细胞增多症的有效治疗选择。