2026年 · 第106卷第23期

A 45-year-old female was seen in Peking Union Medical College Hospital on Feb. 21, 2025 due to "abdominal distension and vomiting for 3 months". From January 2025, the patient experienced abdominal distension, the efficacy of oral omeprazole and mosapride was poor, the symptoms gradually worsened and vomiting after eating occurred, the serum albumin decreased from 39 g/L to 16 g/L, and the body weight decreased by 10 kg. Nothing special in the past. Physical examination: Left abdominal and periumbilical tenderness. Gastroscopy: chronic superficial atrophic gastritis. Intestinal ultrasound: The small intestinal wall of groups 2 to 4 was thickened, the thickest part was 0.7 cm, and a strip of blood flow signal was seen in the intestinal wall. Abdominopelvic enhanced CT: Multiple intestinal wall thickening of the small intestine in the proximal abdominopelvic cavity, accompanied by mucosal enhancement, and some intestinal lumen slightly dilated (Figure 1, indicated by arrows). Intestinal protein loss imaging: positive, the site of protein loss was the small intestine of group 3. Enteroscopy: Group 3 small intestinal mucosal congestion and edema with segmental annular cavity superficial ulcer covered with white mucus (Figure 2); Mucosal biopsy was performed at the lesion, and pathology showed a large number of plasma-like cell infiltration and powder-stained amorphous material deposition in the mucosa (hematoxylin-eosin staining ×10) (Figure 3, indicated by arrows). Immunohistochemistry: AE1/AE3 (epithelial +), CD3 (scattered +), CD20 (scattered +), CD138 (+), Mum-1 (+), Kappa (+), Lambda (-), Ki-67 (index5%), CD21 (-), CyclinD1 (-), CD19 (partial +), CD56 (-), LCA (+), CD117 (-). Special staining: Congo red (weak +), alcoholized Congo red (weak +), Masson (-). Bone marrow biopsy showed no tumor involvement. The final diagnosis was mucosa-associated lymphoid tissue (MALT) extranodal marginal zone lymphoma with plasmacytoid differentiation and amyloid deposition. The patient was treated with ortuzumab combined with orelabrutinib and lenalidomide. The patient's disease progressed after 3 cycles of treatment; Therefore, the regimen was changed to rituximab combined with bendamustine. After 2 cycles of treatment, the patient's symptoms improved not significantly. During this period, the anorexia gradually worsened and the patient had intermittent fever. The serum albumin was 20 g/L on re-examination, which was only slightly higher than that before treatment. Thereafter, the patient returned to the local area to continue chemotherapy (specific protocol unknown) and was lost to follow-up on 29 Aug 2025. Primary small intestinal MALT extranodal marginal zone lymphoma is rare, and hypoalbuminemia and long intestinal involvement are associated with poor prognosis. In addition to the tumor itself, fibrous stenosis secondary to repeated chronic inflammation adjacent to the tumor can also lead to persistent abdominal pain, vomiting and other symptoms, and the response to drug treatment is poor. Some patients can surgically remove the diseased intestinal segment to improve their quality of life.