This is a rare case, a vice Minister with a lymphoma presenting as an initial GI involvement. The whole story is following:

This is a 62-year-old woman who began having diarrhea (up to 6 times per day, very loose, with no definite blood)for 3-4 months.  The endoscope examination showed duodenum and terminal ileum with edema-like hypertrophic villi/mucosa in a patchy distribution.

Biopsy shows villi are distended by amorphous eosinophilic hyalinized material. Notice, some of the hyalinized material looked like in the lymphatic spaces, but 1) the color of the material is much dense and dark than lymphoid fluid, and 2) the material is associated with lymphocytes and plasma cells.  Initial study included Congo red stain and PAS stain. Congo red was negative. PAS stain was positive.  Subsequently, IgA and IgM immunostains were performed. IgM was strongly positive (shown in Figure 5 below).

Diagnosis: IgM gammopathy involving intestine. Recommend clinical work-out for lymphoid proliferative disorders, including plasmacytoid lymphoma(Waldenstrom macroglobulinemia), lymphoplasmacytic lymphoma, among others.

Subsequently, bone marrow biopsy was performed. It showed 1) marked increase of lymphocytes (numerous small and intermediate to rare large size lymphoid cells with moderate to abundant amounts of pale, agranular cytoplasm.  The nuclear morphology ranges from round to ovoid with evenly distributed condensed, mature appearing chromatin.  Nucleoli are absent), and 2) marked increase of  plasma cells ( display a spectrum of morphologies ranging from typical to slightly enlarged cell size with nucleomegaly, increased N:C ratios and indistinct nucleoli.  Occasional plasma cells demonstrate clear cytoplasmic vacuoles). 

Flow cytometric immunophenotypic studies were performed on portions of the bone marrow aspirate identified a monotypic kappa B-lymphoid and plasma cell population. The B-cells are positive for CD19 and CD20, but are negative for CD10. A subpopulation appears to have variable CD5 expression. The monotypic plasma cells are positive for CD19, CD38, CD52, CD138 and are negative for  CD20 and CD56. These findings support a lymphoplasmacytic neoplasm.

FINAL DIAGNOSIS

            LYMPHOPLASMACYTIC LYMPHOMA INVOLVES BONE MARROW AND INTESTINE.

在独立之前要学习的东西实在是太多了

 答案最终水落石出，让我大跌眼镜！

这样的图像我想大多数会像我一样的视而不见的，可见认真阅片是多么重要呀！

多谢谈老师

 这样的图像我多会视而不见的。

谢谢谈老师！

 玻璃样物质沉积。淀粉样物质？刚果红染色和PAS染色鉴别。

淀粉样变性病？是系统性还是局限性？

患者是否有骨髓瘤病史？

有腹泻症状？

The dense pink material is thicker and darker than lymph fluid. The mixed cells are predominately plasma cells.

More photos.

Notice that the dark dense pink material is mixed with cells. The pink material is within the laminar propria. The details of the cells are shown in the last photo.

 吸收障碍：

（1）乳糜泻

（2）非热带口炎性腹泻

（3）谷蛋白敏感性肠病

（4）热带性口炎性腹泻

（5）Whipple病（肠源性脂肪代谢障碍）

（6）伴有营养吸收障碍的疾病：棘红细胞增多症（无B脂蛋白血症）、血r球蛋白缺乏性口腔炎性腹泻、硬皮病、钩虫等寄生虫感染、疱疹性皮炎、乳糖酶缺乏、恶性肿瘤如恶性淋巴瘤等。

该患者去过墨西哥，不知有否热带性口炎？

 从临床表现看，也有可能是吸收障碍所制。

62岁女性患者最近去过墨西哥。主诉下腹痛10周，近10天来水样大便，每天5-6次。临床怀疑感染性疾病。内镜显示，十二指肠和末段回肠水肿样表现。

 这个十二指肠黏膜活检病理所见如下：

（1）叶状绒毛有萎缩，仅显示部分性萎缩；

（2）淋巴管（乳糜管）有扩张，淋巴液停滞；

（3）有小灶糜烂；

（4）黏膜充血、水肿，炎性细胞浸润不是特别多，没有大量的浆细胞浸润；

（另没见到黏膜下层十二指肠腺，可能是活检小而局限的原因）

以上尤其(1）和（2）的特点，需考虑吸收障碍性疾病。