Colonic lymphoma producing alpha-chain disease protein

Area of Science:

• Immunology
• Gastroenterology
• Oncology

Background:

• Alpha-chain disease typically involves the small intestine, causing villus atrophy and malabsorption.
• This presentation of alpha-chain disease with colonic lymphoma and no malabsorption is unusual.

Observation:

• A 57-year-old male presented with a colonic tumor diagnosed as malignant lymphoma.
• The tumor infiltrated the mesentery, and alpha-chain disease protein was detected in serum, urine, and tumor tissue.
• The alpha-chain disease protein was classified as subclass 1.

Findings:

• The patient received cyclophosphamide and prednisone, showing a positive clinical response.
• Despite treatment, intraabdominal lymphoma with gastric involvement persisted, and serum alpha-chain protein remained detectable.
• This case may represent a distinct variant of alpha-chain disease.

Implications:

• This case expands the known clinical spectrum of alpha-chain disease.
• Highlights the importance of considering alpha-chain disease in non-typical presentations involving lymphoma.
• Suggests potential therapeutic strategies for this rare variant.