CD8-positive indolent T-Cell lymphoproliferative disorder of the gastrointestinal tract: A case report and review of literature

Abstract

Background: Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract (ITLPD-GI), a primary tumor forming in the gastrointestinal (GI) tract, represents a rarely diagnosed clonal T-cell disease with a protracted clinical course.

Case summary: This report presented a 45-year-old male patient with a 6-year history of anal fistula and a more than 10-year history of recurrent diarrhea who was not correctly diagnosed until the occurrence of complications such as intestinal perforation. Postsurgical histopathological analysis, combined with hematoxylin-eosin staining, immunohistochemistry and TCRβ/γ clonal gene rearrangement test, confirmed the diagnosis of CD8+ ITLPD-GI.

Conclusion: Individuals with this scarce lymphoma frequently show non-specific symptoms that are hard to recognize. So far, indolent CD8+ ITLPD-GI has not been comprehensively examined. The current mini-review focused on evaluating indolent CD8+ ITLPD-GI cases based on existing literature and discussing future directions for improved differential diagnosis, detection of genetic and epigenetic alterations, and therapeutic target identification.

Keywords: Case report; Gastrointestinal tract; Immunohistochemistry; Indolent T-cell lymphoproliferative disease; Inflammatory bowel disease.

Figure 1

Magnetic resonance imaging and retroperitoneal B-ultrasound manifestations. A: Thickened small bowel wall and whole colon, with enlarged regional lymph nodes at the mesenteries; B: Retroperitoneal B-ultrasound manifestation: Multiple retroperitoneal lymph nodes are enlarged. The orange arrow points to the largest swollen lymph node; C: Pathological results of lymph node puncture showing the destruction of lymph node structure and diffuse proliferation and infiltration of tumor cells in the paracortical area and medullary sinus.

Figure 2

Endoscopic and pathologic findings of the stomach and intestines. A: Colonoscopy manifestation: multiple ulcers are seen in the distal ileum, colon and rectum, with two large ulcerations each in the distal ileum and sigmoid colon (Orange arrow); B: Gastroscopy shows chronic atrophic gastritis.

Figure 3

Superior mesenteric arteriography and perioperative images. A: Superior mesenteric arteriography shows rupture and hemorrhage of a straight arteriole distal to the ileocolic artery; subsequently, microspring coils are used to embolize the diseased vessels, and repeated angiography shows that the hemorrhagic lesion disappears 5 minutes later (Red box); B: A large amount of yellow-green intestinal fluid in the abdominal cavity and ileum perforation are observed during the operation (Orange arrow).

Figure 4

Immunohistochemical findings. Colonic lymphoid cells undergo immunophenotyping by immunohistochemistry. Lymphoid cells show positivity for CD3, CD5, CD7 and CD8, and negativity for CD4, CD20 and CD56. The Ki-67 proliferative index is low (< 10%). Monoclonal gene rearrangement is determined in T-cell receptor-clonality assay by polymerase chain reaction.

Figure 5

TCRβ/γ clonal gene rearrangement. TCRβ/γ clonal gene rearrangement test by PCR and Capillary Electrophoresis. The results shows positivity for C511-BA, C511-BB, and negativity for C511-GA, C511-BC and C511-GB and C511-D.