Kai Keita, Miyahara Masaharu, Tokuda Yasunori, Kido Shinich, Masuda Masanori, Takase Yukari, Tokunaga Osamu
Keita Kai, Masanori Masuda, Yukari Takase, Osamu Tokunaga, Departments of Pathology and Microbiology, Saga University Faculty of Medicine, Saga 849-8501, Japan.
World J Clin Cases. 2013 Aug 16;1(5):176-80. doi: 10.12998/wjcc.v1.i5.176.
A 73-year-old Japanese woman was hospitalized for detailed examination of nausea, diarrhea and loss of appetite. Atypical erosion in the ileum was found on endoscopy. Biopsy of this erosion showed proliferation of cells containing numerous Russell bodies. Differential diagnoses considered were Russell body enteritis, crystal-storing histiocytosis, Mott cell tumor, immunoproliferative small intestinal disease (IPSID) and mucosa-associated lymphoid tissue (MALT) lymphoma. The cells containing prominent Russell bodies showed diffuse positivity for CD79a and CD138, but negative results for CD20, CD3, UCHL-1, CD38 and CD68. Russell bodies were diffusely positive for lambda light chain, but negative for kappa light chain, and immunoglobulin (Ig) G, IgA and IgM. Based on these findings, Russell body enteritis, crystal-storing histiocytosis and IPSID were ruled out. As the tumor formed no mass lesions and was restricted to the gastrointestinal tract, MALT lymphoma with extensive plasma cell differentiation was finally diagnosed. The patient showed an unexpectedly aggressive clinical course. The number of atypical lymphocytes in peripheral blood gradually increased and T-prolymphocytic leukemia (T-PLL) emerged. The patient died of T-PLL 7 mo after admission. Autopsy was not permitted.
一名73岁的日本女性因恶心、腹泻和食欲不振入院进行详细检查。内镜检查发现回肠有非典型糜烂。对该糜烂进行活检显示，含有大量拉塞尔小体的细胞增生。考虑的鉴别诊断包括拉塞尔小体肠炎、贮晶组织细胞增多症、莫特细胞瘤、免疫增殖性小肠疾病（IPSID）和黏膜相关淋巴组织（MALT）淋巴瘤。含有明显拉塞尔小体的细胞对CD79a和CD138呈弥漫性阳性，但对CD20、CD3、UCHL-1、CD38和CD68呈阴性。拉塞尔小体对λ轻链呈弥漫性阳性，但对κ轻链以及免疫球蛋白（Ig）G、IgA和IgM呈阴性。基于这些发现，排除了拉塞尔小体肠炎、贮晶组织细胞增多症和IPSID。由于肿瘤未形成肿块病变且局限于胃肠道，最终诊断为具有广泛浆细胞分化的MALT淋巴瘤。患者表现出意想不到的侵袭性临床病程。外周血中非典型淋巴细胞数量逐渐增加，出现了T原淋巴细胞白血病（T-PLL）。患者入院7个月后死于T-PLL。未进行尸检。
