Primary small-bowel adult T-cell leukemia/lymphoma with gastric AL amyloidosis

An 86-year-old man was admitted because of small-bowel wall thickening and mesenteric lymphadenopathy. Neither peripheral lymphadenopathy nor hepatosplenomegaly was noted. The leukocyte count was 7930/mm³, and no abnormal lymphocytes were found. The result of testing for serum antihuman T-lymphotropic virus type 1 (HTLV-1) antibody was positive. A bone marrow biopsy was normal without evidence of lymphoid infiltration. Small-bowel enteroclysis demonstrated a narrowing, edematous lesion with multiple ulcerations in the jejunum ([Fig. 1]). Capsule endoscopy ([Fig. 2]) and double-balloon endoscopy ([Fig. 3]) showed enlarged Kerckring folds with annular and irregularly shaped shallow ulcers. Biopsy specimens from the ulcers revealed diffuse infiltration of medium to large pleomorphic lymphoid cells with CD3 +, CD4 +, CD5 +, CD25 +, CCR4 +, FoxP3 +, CD8 –, CD20 –, CD56 –, and TIA1 – immunophenotype ([Fig. 4]). Biopsy specimens from the stomach, duodenum, ileum, and colon showed no lymphoma cells. Fluorodeoxyglucose F 18 positron emission tomography showed abnormal uptake in the jejunal mass, wide areas of mesentery, and para-aortic lymph nodes. Esophagogastroduodenoscopy with chromoendoscopy revealed granular stomach mucosa and swollen folds in the corpus, with pan-atrophic gastritis ([Fig. 5]). Biopsy from the granular mucosa and folds showed massive amyloid deposits of homogeneous acidophilic substances in the submucosa and around the muscularis mucosae ([Fig. 6]), which were immunohistochemically positive for λ light chain. Amyloid deposits were not observed in any specimen taken from the duodenum, jejunum, ileum, and colon. Therefore, the patient’s diagnosis was stage IIE (Lugano classification) small-bowel adult T-cell leukemia/lymphoma (ATLL) accompanied by gastric AL amyloidosis.

Fig. 1 Primary small-bowel adult T-cell leukemia/lymphoma with gastric AL amyloidosis. Small bowel enteroclysis demonstrates a narrowing edematous lesion with multiple ulcerations in the jejunum.

Fig. 2 Capsule endoscopy shows multiple ulcers with enlarged Kerckring folds in the jejunum.

Fig. 3 Double-balloon endoscopy reveals enlarged Kerckring folds with annular and irregularly shaped shallow ulcers in the jejunum.

Fig. 4 Histology of a biopsy specimen from the jejunal ulcer showing diffuse infiltration of medium to large pleomorphic lymphoid cells with CD4 +, CD25 +, CD8 – immunophenotype (magnification × 400).

Fig. 5 Esophagogastroduodenoscopy reveals granular mucosa and swollen folds in the corpus, with pan-atrophic gastritis.

Fig. 6 Histology of a biopsy specimen from the granular gastric mucosa showing massive amyloid deposits of homogeneous acidophilic substances in the submucosa and around the muscularis mucosae (Dylon stain, magnification × 100).

Although systemic ATLL often involves the small bowel [1] [2] [3], primary small-bowel ATLL is extremely rare [4] [5]. Endoscopic findings of small-bowel involvement with ATLL have included multiple polypoid or reddish elevated lesions [2] [3]. However, we believe that the enlarged Kerckring folds with annular or irregularly shaped shallow ulcers observed in our case may be characteristic in primary small-bowel ATLL.

Endoscopy_UCTN_Code_CCL_1AC_2AC

• References

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