Experience and Outcome of Patients with Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL) over 8 Years

Introduction: NLPHL is an uncommon variant of Hodgkin lymphoma (HL) accounting for 5% incidence of all HL diagnoses. NLPHL represents a more indolent disease than classical HL, and is therefore managed uniquely. Unlike classical Hodgkin lymphoma (cHL), NLPHL shows CD20 immunoreactivity. Given the rarity of the disease, optimal management is unclear.

Here we present our retrospective clinical experience of nine cases of NLPHL diagnosed over the last 8 years at our institution.

Results: The median age at diagnosis was 45 years. Eight of the nine patients were males. The youngest patient was 21 years old and the oldest patient at diagnosis was 58 years old. One patient had Stage I, six had stage II and two patients had Stage-III disease. No patient had bone marrow involvement and only two patients had B symptoms. Interestingly, two sets of brothers diagnosed at different intervals were identified among this group of patients.

Three out of seven patients with Stage I/II disease received radiation as the primary modality of treatment. Four patients received chemotherapy only and two patients received combined modality treatment( chemotherapy followed by radiation). Only the youngest patient received R-CVP (rituximab, cyclophosphamide, vincristine, and prednisone) to avoid anthracycline related long term toxicity. A total of four patient received R-CHOP, two of these also received involved site radiation therapy (ISRT).One patient received six cycles of AVD (doxorubicin, vincristine and dacarbazine).

All the patients achieved complete response (CR) and durable remission except one patient. That particular patient had stage III NLPHL at diagnosis and was treated with R-CHOP initially. Three years later on relapse, he had T-cell rich large B cell lymphoma (TCRBCL) and is currently on a clinical trial with pembrolizumab and remains in complete remission.

Conclusions: As has been the experience with other institutions, the majority of the cases of NLPHL at our institution have presented with early stage disease. Treatments have included R-CHOP regimens in most of the cases with durable remission in the majority. Although R-CVP is not the standard regimen for NLPHL, the one young patient who had stage III disease at diagnosis and received R-CVP, achieved CR and remains disease free five years later. This highlights the importance of non-anthracycline chemotherapy regimens as an option for particularly young or older frail patients.

NLPHL occurs with higher than expected frequency among first-degree family members of patients with NLPHL as demonstrated in the Finnish Cancer Registry study reported in JCO 2013. Interestingly, in our small cohort, there were two sets of brothers identified with NLPHL, diagnosed metachronously. Unlike cHL, late relapse can occur as well as a propensity to transform to an aggressive B-cell Non-Hodgkin lymphoma.