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Primary gastrointestinal anaplastic large cell lymphoma.
Yi-Ying Lee1, Katsuyoshi Takata2, Ren-Ching Wang3
1Department of Pathology, Chi-Mei Medical Center, Liouying, Taiwan.
Pathology
|July 12, 2017
Summary
Primary gastrointestinal anaplastic large cell lymphoma (GI-ALCL) is rare. Anaplastic lymphoma kinase (ALK) negative GI-ALCL has a poor prognosis, while ALK positive GI-ALCL shows a good prognosis.
Area of Science:
- Oncology
- Hematology
- Gastroenterology
Background:
- Primary gastrointestinal anaplastic large cell lymphoma (GI-ALCL) is a rare condition.
- It predominantly affects older individuals and frequently involves the stomach and small intestine.
Purpose of the Study:
- To characterize the clinical features, molecular profile, and outcomes of GI-ALCL.
- To compare the prognosis of anaplastic lymphoma kinase (ALK) positive versus ALK negative GI-ALCL.
Main Methods:
- Case series of eight patients with GI-ALCL.
- Literature review of existing GI-ALCL cases.
- Immunohistochemistry for CD30 and ALK protein expression.
- In situ hybridization for Epstein-Barr virus and DUSP22/IRF4 gene translocation.
Main Results:
- The median age of patients was 61.5 years, with stomach and small intestine as common sites.
- Anaplastic lymphoma kinase (ALK) protein expression was observed in 25% of cases.
- ALK-negative GI-ALCL had a 5-year overall survival of 40%, compared to 100% for ALK-positive cases.
Conclusions:
- GI-ALCL is characterized by a low rate of ALK expression and a poor prognosis in ALK-negative cases.
- ALK-positive GI-ALCL, though rare, appears to have a favorable prognosis.
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