[Clinical Observation of Five Pediatric-type Follicular Lymphoma in Adult]

Overview

Journal Zhonghua Xue Ye Xue Za Zhi

Specialty Hematology

Date 2019 Jun 18

PMID 31207704

Authors

X Y Du

R Huang

L Cao

W Wu

Z Wang

H Y Zhu

L Wang

L Fan

W Xu

J Y Li

Affiliations

Soon will be listed here.

Abstract

To investigate the characteristics in pathological diagnosis, clinical features, treatment and prognosis of adult patients with pediatric-type follicular lymphoma (PTFL) . 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. 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. 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.

References

Quintanilla-Martinez L, Sander B, Chan J, Xerri L, Ott G, Campo E . Indolent lymphomas in the pediatric population: follicular lymphoma, IRF4/MUM1+ lymphoma, nodal marginal zone lymphoma and chronic lymphocytic leukemia. Virchows Arch. 2015; 468(2):141-57. DOI: 10.1007/s00428-015-1855-z. View

Federico M, Luminari S, Dondi A, Tucci A, Vitolo U, Rigacci L . R-CVP versus R-CHOP versus R-FM for the initial treatment of patients with advanced-stage follicular lymphoma: results of the FOLL05 trial conducted by the Fondazione Italiana Linfomi. J Clin Oncol. 2013; 31(12):1506-13. DOI: 10.1200/JCO.2012.45.0866. View

Agrawal R, Wang J . Pediatric follicular lymphoma: a rare clinicopathologic entity. Arch Pathol Lab Med. 2009; 133(1):142-6. DOI: 10.5858/133.1.142. View

Martin-Guerrero I, Salaverria I, Burkhardt B, Szczepanowski M, Baudis M, Bens S . Recurrent loss of heterozygosity in 1p36 associated with TNFRSF14 mutations in IRF4 translocation negative pediatric follicular lymphomas. Haematologica. 2013; 98(8):1237-41. PMC: 3729904. DOI: 10.3324/haematol.2012.073916. View

Swerdlow S . Pediatric follicular lymphomas, marginal zone lymphomas, and marginal zone hyperplasia. Am J Clin Pathol. 2005; 122 Suppl:S98-109. DOI: 10.1309/4BKNAKE4D7CT3C1B. View

Liu Q, Salaverria I, Pittaluga S, Jegalian A, Xi L, Siebert R . Follicular lymphomas in children and young adults: a comparison of the pediatric variant with usual follicular lymphoma. Am J Surg Pathol. 2012; 37(3):333-43. PMC: 3566339. DOI: 10.1097/PAS.0b013e31826b9b57. View

Heller K, Teruya-Feldstein J, La Quaglia M, Wexler L . Primary follicular lymphoma of the testis: excellent outcome following surgical resection without adjuvant chemotherapy. J Pediatr Hematol Oncol. 2004; 26(2):104-7. DOI: 10.1097/00043426-200402000-00007. View

Schmidt J, Ramis-Zaldivar J, Nadeu F, Gonzalez-Farre B, Navarro A, Egan C . Mutations of are frequent in pediatric-type follicular lymphoma and result in ERK pathway activation. Blood. 2017; 130(3):323-327. PMC: 5520474. DOI: 10.1182/blood-2017-03-776278. View

Oschlies I, Salaverria I, Mahn F, Meinhardt A, Zimmermann M, Woessmann W . Pediatric follicular lymphoma--a clinico-pathological study of a population-based series of patients treated within the Non-Hodgkin's Lymphoma--Berlin-Frankfurt-Munster (NHL-BFM) multicenter trials. Haematologica. 2009; 95(2):253-9. PMC: 2817028. DOI: 10.3324/haematol.2009.013177. View

10.

Ozawa M, Bhaduri A, Chisholm K, Baker S, Ma L, Zehnder J . A study of the mutational landscape of pediatric-type follicular lymphoma and pediatric nodal marginal zone lymphoma. Mod Pathol. 2016; 29(10):1212-20. PMC: 5047957. DOI: 10.1038/modpathol.2016.102. View

11.

Schmidt J, Gong S, Marafioti T, Mankel B, Gonzalez-Farre B, Balague O . Genome-wide analysis of pediatric-type follicular lymphoma reveals low genetic complexity and recurrent alterations of TNFRSF14 gene. Blood. 2016; 128(8):1101-11. PMC: 5000845. DOI: 10.1182/blood-2016-03-703819. View

12.

Louissaint Jr A, Schafernak K, Geyer J, Kovach A, Ghandi M, Gratzinger D . Pediatric-type nodal follicular lymphoma: a biologically distinct lymphoma with frequent MAPK pathway mutations. Blood. 2016; 128(8):1093-100. PMC: 5000844. DOI: 10.1182/blood-2015-12-682591. View

13.

Lorsbach R, Moore J, Banks P, Hasserjian R, Sandlund J, Behm F . Clinicopathologic analysis of follicular lymphoma occurring in children. Blood. 2002; 99(6):1959-64. DOI: 10.1182/blood.v99.6.1959. View

14.

Frizzera G, MURPHY S . Follicular (nodular) lymphoma in childhood: a rare clinical-pathological entity. Report of eight cases from four cancer centers. Cancer. 1979; 44(6):2218-35. DOI: 10.1002/1097-0142(197912)44:6<2218::aid-cncr2820440634>3.0.co;2-d. View

15.

Al-Tourah A, Gill K, Chhanabhai M, Hoskins P, Klasa R, Savage K . Population-based analysis of incidence and outcome of transformed non-Hodgkin's lymphoma. J Clin Oncol. 2008; 26(32):5165-9. DOI: 10.1200/JCO.2008.16.0283. View

16.

Louissaint Jr A, Ackerman A, Dias-Santagata D, Ferry J, Hochberg E, Huang M . Pediatric-type nodal follicular lymphoma: an indolent clonal proliferation in children and adults with high proliferation index and no BCL2 rearrangement. Blood. 2012; 120(12):2395-404. DOI: 10.1182/blood-2012-05-429514. View

17.

Koo M, Ohgami R . Pediatric-type Follicular Lymphoma and Pediatric Nodal Marginal Zone Lymphoma: Recent Clinical, Morphologic, Immunophenotypic, and Genetic Insights. Adv Anat Pathol. 2017; 24(3):128-135. DOI: 10.1097/PAP.0000000000000144. View

18.

Ribeiro R, Pui C, MURPHY S, Shuster J, Hvizdala E, Falletta J . Childhood malignant non-Hodgkin lymphomas of uncommon histology. Leukemia. 1992; 6(8):761-5. View

19.

Swerdlow S, Campo E, Pileri S, Lee Harris N, Stein H, Siebert R . The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016; 127(20):2375-90. PMC: 4874220. DOI: 10.1182/blood-2016-01-643569. View

20.

Attarbaschi A, Beishuizen A, Mann G, Rosolen A, Mori T, Uyttebroeck A . Children and adolescents with follicular lymphoma have an excellent prognosis with either limited chemotherapy or with a "Watch and wait" strategy after complete resection. Ann Hematol. 2013; 92(11):1537-41. DOI: 10.1007/s00277-013-1785-2. View