Clinical analysis of 14 cases of rare non-Hodgkin lymphoma in children

Abstract

Objective To analyze the clinical characteristics and prognosis of 4 rare types of non-Hodgkin lymphoma(NHL) in children, and to discuss the progress in treatment. Methods Clinical data of 14 patients with rare types of NHL at Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine between January 2004 and December 2014 were retrospectively analyzed, and their clinical features, treatment and prognosis were discussed. Results Fourteen cases were reported including 6 subcutaneous panniculitis-like T-cell lymphoma(SPTCL), 3 hydroa vacciniforme-like cutaneous lymphoma(HVLL), 2 pediatric follicular lymphoma(PFL) and 3 extranodal NK/T-cell lymphoma, and nasal type(ENKTL). Ten patients (71.4%) primarily presented with skin lesions and underwent a long course of illness before they were finally diagnosed (the median was 10 months), 71.4%(10/14 cases) of them associated with fever and 50.0%(7/14 cases) with liver and spleen enlargement, and no evidence of central nervous system (CNS) and bone marrow (BM) involvement was observed, while 28.6% patients (4/14 cases) had more than two lines' abnormalities in peripheral blood examination.Since there were no standard treatment guidelines, most patients received CHOP (Cyclophosphamide+ Adriamycin+ Vincristine+ Prednisone) and/or mature B-cell NHL-like therapy, and 50.0%(7/14 cases) of them received interferon therapy in addition, while 1 patient received allogeneic hematopoietic stem cell transplantation after recurrence.The complete remission was achieved in 71.4%(10/14 cases) of all the patients.Two of them died, 3 lost follow-up, and 1 relapsed.The 3-year overall survi-val and event free survival rates were 0.84 and 0.57, respectively, after a median follow-up of 26 months (range 12-64 months). Conclusions Pediatric rare types of NHL show atypical clinical manifestation, low incidence of CNS/BM infiltration and long course.It is hard to make pathological diagnosis and differentiation.It is also inappropriate to apply the commonly used staging system to these rare types of NHL.No standard treatment has been found by now.SPTCL, HVLL and PFL have relatively good outcomes when treated with mature B-cell NHL-type therapy plus interferon therapy. Key words: Subcutaneous panniculitis-like T-cell lymphoma; Hydroa vacciniforme-like cutaneous lymphoma; Pediatric follicular lymphoma; Extranodal NK/T-cell lymphoma, nasal type; Child