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Published on: March 26, 2018
[The leukemia-lymphoma syndrome with gastrointestinal involvement in childhood. A case report]
A Busca1, M Giacchino, R Cappelletto
1Clinica Pediatrica III, Università di Torino.
Insights
Acute lymphoblastic leukemia rarely affects the gastrointestinal tract in children. This case highlights successful treatment of leukemia-lymphoma syndrome involving the CNS and GI tract, achieving remission.
Area of Science:
- Pediatric Oncology
- Hematology
- Gastrointestinal Oncology
Background:
- Acute lymphoblastic leukemia (ALL) is a common childhood cancer.
- Gastrointestinal (GI) tract involvement at diagnosis is rare in pediatric ALL.
- Leukemia-lymphoma syndrome is an aggressive variant requiring specific management.
Observation:
- A pediatric patient presented with symptoms suggestive of both leukemia and lymphoma.
- Diagnostic workup revealed acute lymphoblastic leukemia with concurrent central nervous system (CNS) and GI tract involvement.
- This presentation is unusual, as GI tract involvement is an uncommon initial manifestation of pediatric ALL.
Findings:
- The patient received intensive combination chemotherapy tailored to the leukemia-lymphoma syndrome.
- The treatment regimen was effective in managing the widespread disease.
- The child achieved a continuous complete remission off-therapy 29 months post-diagnosis.
Implications:
- This case underscores the importance of considering rare presentations of pediatric ALL.
- Aggressive chemotherapy can lead to long-term remission even in complex cases with CNS and GI involvement.
- Further research into optimal treatment strategies for leukemia-lymphoma syndrome with extranodal involvement is warranted.
Abstract:
The gastrointestinal tract is an uncommon site of presentation for acute lymphoblastic leukemia in children. We report a child who developed a leukemia-lymphoma syndrome with central nervous system and gastrointestinal tract involvement at the diagnosis. The patient received an intensive combination chemotherapy and is currently off-therapy in continuous complete remission 29 months after the diagnosis.

