Clinical observation of five pediatric-type follicular lymphoma in adult
10.3760/cma.j.issn.0253-2727.2019.05.009
- VernacularTitle: 成人儿童型滤泡淋巴瘤五例临床观察
- Author:
Xinyi DU
1
;
Rong HUANG
;
Lei CAO
;
Wei WU
;
Zhen WANG
;
Huayuan ZHU
;
Li WANG
;
Lei FAN
;
Wei XU
;
Jianyong LI
Author Information
1. Department of Hematology, the First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Collaborative Innovation Center for Cancer Personalized Medicine, Nanjing 210029, China
- Publication Type:Journal Article
- Keywords:
Pediatric-type follicular lymphoma;
Adult;
Disease attributes;
Treatment;
Prognosis
- From:
Chinese Journal of Hematology
2019;40(5):393-397
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the characteristics in pathological diagnosis, clinical features, treatment and prognosis of adult patients with pediatric-type follicular lymphoma (PTFL) .
Methods:The clinical and pathological features, laboratory examination, diagnosis and treatment, follow-up results of 5 adult PTFL patients admitted in Jiangsu Province Hospital were retrospectively analyzed, and literature review was conducted in combination with related reports.
Results:All 5 patients developed PTFL in their adulthood with a median age of 22 years old (15-33 years) . The initial inanifestation of the disease was local painless lymphadenopathy with no fever, night sweats, emaciation or other systemic B symptoms. Pathological characteristics including typical large follicular structures and high proliferation index were found. Meanwhile, additional clonal rearrangement of immunoglobulin heavy chain gene was observed. However, there was no BCL-2 expression in histochemistry as well as BCL-2 gene abnormality in fluorescence in situ hybridization among these PTFL patients. These adult PTFL patients were all in stage Ⅰ-Ⅱ of the disease. For treatment, they were only treated with local surgical excision after diagnosis while didn’t receive subsequent local radiotherapy or systemic immunochemotherapy. During a median follow-up of 27 months, the 5 cases of PTFL kept in a state of sustained complete remission.
Conclusion:Adult-onset PTFL is characterized by high pathological proliferation index, while no BCL-2 expression or BCL-2 gene abnormality. Besides, PTFL is clinically manifested as a localized disease that can achieve a quite good prognosis through local surgical intervention. The aforementioned attributes of PTFL are distinctly different from classic adult follicular lymphoma.
