Anaplastic lymphoma kinase-negative anaplastic large cell lymphoma masquerading as Behcet's disease: A case report and review of literature

Background: Anaplastic large cell lymphoma (ALCL) is a CD30-positive T cell lymphoma, a rare type of non-Hodgkin lymphoma. The current World Health Organization classification system divides ALCLs into anaplastic lymphoma kinase (ALK)-positive and ALK-negative groups. ALCL rarely presents in the gastrointestinal tract.

Case summary: A 54-year-old male was admitted to the department of gastroenterology for abdominal pain. He presented with lower abdominal pain, diarrhea and recurrent oral and penile ulcers. He was misdiagnosed with Behcet's disease and treated with prednisone. But after one month, he was hospitalized in another hospital for reexamination. This time, the lesion on the penis was biopsied for histological examination. The final pathological diagnosis was ALCL, ALK-negative. The patient was treated with cyclophosphamide, doxorubicin, vincristine, prednisolone chemotherapy. However, he died within one month.

Conclusion: Gastrointestinal ALCL needs to be considered in the differential diagnosis to avoid delaying treatment. Repeated biopsy is the most important for early diagnosis and treatment.