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Published on: February 2, 2014
[Hydroa vacciniforme-like lymphoma: two cases report and literatures review]
Ling Zhou1, Xue-ju Xu, Yuan Zhang
1Department of Pediatric Hematology and Oncology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.
Insights
Hydroa vacciniforme-like lymphoma (HVLL) in children presents with unique skin rashes. Diagnosis relies on biopsy and immunohistochemistry, with prognosis and treatment still under investigation.
Area of Science:
- Pediatric Oncology
- Dermatology
- Hematology
Background:
- Hydroa vacciniforme-like lymphoma (HVLL) is a rare condition requiring further understanding.
- This study investigates two pediatric cases to elucidate disease characteristics.
Observation:
- Two pediatric patients presented with polymorphous rashes on limbs and craniofacial areas.
- Pathological examination revealed atypical lymphocytic infiltration in the dermis and subcutaneous tissue.
- Immunohistochemistry confirmed T/NK cell infiltration and Epstein-Barr virus encoded small RNA (EBER) positivity.
Findings:
- Case 1, treated with chemotherapy, showed disease progression.
- Case 2, managed with symptomatic treatment, experienced gradual reduction and complete resolution of skin lesions within two months.
Implications:
- HVLL diagnosis is primarily based on skin biopsy and immunohistochemistry.
- Current treatment strategies lack specificity, and prognosis requires further research.
- Understanding HVLL's clinical course and response to treatment is crucial for pediatric patients.
Objective:
Two children with hydroa vacciniforme-like lymphoma (HVLL) were reported for a better understanding of this disease.
Methods:
The clinical manifestation, pathological characteristics, therapeutic outcomes of two children with HVLL were analyzed and presented by compared with what described in literatures.
Results:
Two children were girls, who treated firstly in the hospital in May 2012, July 2012 and their duration were 1 years, more than 10 years respectively. Their clinical manifestations were both limbs and craniofacial polymorphous rashes. Pathological findings revealed that the dermis and subcutaneous tissue were profiled by atypical lymphocytic infiltration. Immunohistochemistry showed that the infiltration of cells from T/NK cell, and Epstein-Barr virus encoded small RNA (EBER)(+). Case 1 was treated with chemotherapy, but her condition continued to deteriorate. Case 2 just received symptomatic treatment, her skin lesions gradually reduced and rash disappeared completely 2 months later.
Conclusion:
HVLL is found with special clinical manifestation, its diagnosis mainly depend on skin biopsy and immunohistochemistry, there is no specific treatment method now, and its prognosis still needs further research.
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