Nodular Lymphocyte Predominant Hodgkin Lymphoma. Experience in a Single Institution in Argentina

Background: Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL) is a rare subtype of Hodgkin Lymphoma (HL) that represents approximately 5% of HL and discloses a distinct clinical-pathological features with an overall good prognosis. Treatment strategies have been based on protocols for HL. Selected patients can be treated with surgery alone.

Objective: Our aim was to describe the clinical characteristics, management and outcome of patients with NLPH.

Methods: Between March 2000 and June 2016, 16 patients (14 M/2 F), median age: 9.8 (range 4.9-14.5) years, were consecutively diagnosed in our institution. Initial chemotherapy schedules were ABVD (Doxorubicin, Bleomycin, Vinblastine and Dacarbazine), COPP/ABVD or AV-PC (Adriamycin/Vincristine/Prednisone/Cyclophosphamide) were administered to 14 patients. When response was partial or null, additional chemotherapy was administered, IF-RT, Anti CD20 and/or HDC/Autologous SCT. A watch-and-wait strategy after lymph node complete surgery was used in two cases when this strategy became trustworthy.

Results: Fourteen patients (87.5%) showed nodal compromise being cervical localization the most frequent localization. Thirteen patients (81.2%) presented localized disease (stage IA: 8, stage IB: 1, stage IIA:4) and 3 (18.7%) advanced disease (stage IIIA:1, stage IVA:1, IVB:1). Two patients had B symptoms. Two IA stage cases (12.5%) had complete node resection. Five patients (31.2%) received 4 cycles of ABVD. One patient (6.2%) received COPP/ABVD. Five patients (31,2%) received 3 cycles of AV-PC. One patient (6.2%) received 6 cycles of AV-PC plus 4 doses of Rituximab. One patient (6.2%) presented progressive disease after 3 cycles of ABVD, additional chemotherapy was administered and HDC/Autologous SCT. Three (18%) patients received IF-RT plus chemotherapy. One patient is still on treatment. Response to treatment was: CR in 12 pts (75%), Partial-response: 2 pts (12.5%), NR: 1 pts (6.2%). Median follow up: 58.4 (range 2-89) months. All patients remain in CR. Conclusion: Although the management of NLPHL was not uniform in our retrospective analysis, the results were promising. Watch and wait strategy plays an important role to avoid adverse effects associated with therapy. Tailored therapy according to staging and disease response, seemed to be a good strategy in our setting.