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DOI: 10.1055/s-0040-1701854
Nodular Lymphocyte Predominant Hodgkin Lymphoma - experience of Polish Pediatric Leukemia/Lymphoma Study Group
Publication History
Publication Date:
18 March 2020 (online)
Introduction Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLP-HL) is a rare clinical entity. In order to investigate NLP-HL clinical course and treatment a survey was performed within Polish Pediatric Leukemia/Lymphoma Study Group participating centers.
Methods Questionnaire was sent to all participating centers and data regarding patients, diagnosis, treatment and treatment results were gained. Analysis of collected data was performed.
Results From 2010 to 2019, 28 pediatric patients with NLP-HL were registered in Poland. Two patients with inherited immune deficiencies and 3 patients who did not complete treatment were excluded from analysis. Age of patients was 5,5 – 17,8 (median: 12,2) years. NLP-HL occurred mainly in males (n=21). All stages of disease were observed but stage I (n=7) and stage II (n=10) were predominating. In most of patients (n=15) supradiaphragmatic localization was observed while mediastinal involvement was observed only in 2 patients.
7 patients presented with localized, resectable disease. In 3 patients total primary resection was performed. One of these patients relapsed and was treated with CVP chemotherapy. Two patients received further chemotherapy after total resection. Two patients were resected incompletely thus underwent CVP chemotherapy. No other relapses were observed.
Fourteen patients presented with unresectable disease, of these 8 received CVP chemotherapy, and 6 were treated with other chemotherapy regimens, three relapses were observed and these patients were further treated with chemotherapy and rituximab. One patient underwent auto-SCT. All patients remain alive. Three-years disease-free survival was 81%.
Conclusion NLP-HL treatment result are consistent with results noted in other countries, but still there is a need for improvement and coherent treatment of NLP-HL patients in Poland. Focus has to be made on primary resection eligible patients. In higher stages of disease consensus on treatment has worked out as many different chemotherapy schedules were in use.
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