Spontaneously Relapsing Clonal, Mucosal Cytotoxic T-cell Lymphoproliferative Disorder: Case Report And Review Of The Literature
Ranheim, E. A., Jones, C., Zehnder, J. L., Warnke, R., & Yuen, A. (2000). spontaneously relapsing clonal, mucosal cytotoxic T-cell lymphoproliferative disorder: case report and review of the literature. The American journal of surgical pathology, 24(2), 296–301. https://doi.org/10.1097/00000478-200002000-00017
View Original Source →Abstract
Primary T-cell lymphoma of the gastrointestinal tract is a rare and usually aggressive disorder that may be associated with celiac disease. The authors describe a unique case of a clonal proliferation of CD8+ T cells involving the oral mucosa, ileum, and colon of a 35-year-old man that has regressed spontaneously and recurred numerous times over a 9-year period without treatment. The patient's symptoms were limited to occasional rectal bleeding and recurring painful oral ulcers. Within the intestine, these collections of small T cells induced minimal architectural distortions and did not show extensive epitheliotrophism. Polymerase chain reaction and sequencing analyses revealed that the identical T-cell clone has been present for more than 9 years and in different mucosal locations in this patient. This may represent a unique T-cell lymphoproliferative process akin to a mucosal counterpart of lymphomatoid papulosis of the skin.
Case Details
Disease Location
Oral cavity, lung, bowel, spleen
Personal Characteristics
35 -year-old male, asian, non-smoker, occasional alcohol use 9 year history of recurrent oropharyngeal ulcers and rectal bleeding the oral uclers recurred approx. Every 6 months and resolved without treatment over a 1-2 months span no family history of leukemia, lymphoma or other neoplasia
Clinical Characteristics
Colonoscopies in 1990 and 1994, secondary to rectal bleeding revealed small oozing erosions of the mucosa of the colon; initial histologic findings revealed minimal, focal acute colitis and a prominent lymphocytic inflammatory infiltrate repeated colonic biopsies in july 1998 revealed mild acute colitis and a prominent lymphoid infiltrate that showed clonal tcr gene rearrangement by polymerase chain reaction analysis. Bone marrow biopsy was negative for lymphoma he denied fever, cills, night sweats, vomiting, fatigue, or weight loss. On examination, he appeared healthy without evidence of lymphadenopathy. The skin was unremarkable the posterior pharynx contained a number of green-tinged, fungating ulcerations in the area of the uvula, several partially healed ulcers were present, crusted over with black, scabbed mucosa CT revealed an ill-defined right upper lobe density in the lung, consistent with chronic granulomatous disease, diffuse thickening of the small bowel valvulae, mildly enlarged mesenteric lymph nodes, and mild splenomegaly a biospy of the palate lesion was taken in oct 1998 immunohistochemical and molecular analysis was performed on specimens from the sigmoid colon (1990), the cecum, ileum, and rectum (1994), the right, left, and sigmoid colon (1998), and palate (1998). Initial biopsy from 1990 had been interpreted as nonspecific colitis with petechial hemorrhage at an outside community architecture without ulceration or notable acute inflammation. The lamina propria was filled with a mixed infiltrate of lymphocytes, eosinophils, and plasma cells with the lymphocytic component extending into the submucosal tissue. The lymphocytes in the submucosa showed increased amounts of clear cytoplasm and slightly angulated nuclei without marked cytologic atypical. Immunostaining showed a majority of cells to be CD3+ and CD8+ lymphocytes with a single small follicle of residual normal b-cells intestinal biopsies from 1994 and 1998 showed similar findings with prominent lymphocytic infiltrates consisting primarily of small CD3 and 8 positive t-cells in the terminal ileum, cecum, right and left colon and rectum. Scattered CD4+ cells were in the colonic biopsies from 1998 but these accounted for <10% of the lymphoid cells. There was normal mucosal architecture in all locations. Only focal infiltration of the epithelium by small t-cells was noted. There was no appearance of increased intraepithelial lymphocytes in affected tissues. Focal acute inflammation was evident, but frank crypt abscesses or granulomas were absent the palate biopsy from 1998 showed a submucosal infiltrate of small to mediium lymphoid cells with clear cytoplasm and slightly angulated nuclei. Rare larger cells with prominent nucleoli were present. Lymphocytes were also scattered in the squamous epithelium, there was evident ulceration and necrosis of surface epithelium. Large majority of cells were CD3 and 8 positive t cells. There was no expression of NK marker CD56, 4, or cytotoxic granule marker tia-1, but there was stain for pan t-cell marker CD5 and tcr-beta chain. There was no evidence of ebv eber-1 RNA clonal tcr-gamma rearrangement was detected in all cases (there is more about isolated DNA bands in the article, want sure if necessary thus left out) the same t-cell clone persisted for more than 9 years in multiple locations
Remission Characteristics
Proliferation would regress and recur over a 9-year period nothing major reported about the regressional periods
Treatment & Mechanisms
Proposed Remission Mechanisms
No major mechanims proposed
Clinical Treatment
None reported
Non-Clinical Treatment
None reported