How I treat nodular lymphocyte predominant Hodgkin lymphoma

Abstract

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is an uncommon entity that, in contrast to classical Hodgkin lymphoma (cHL), universally expresses CD20, a hallmark of the disease. The majority of the patients present with early-stage disease, and treatment with local radiation provides excellent disease control and overall survival (OS). For locally extensive or advanced stages, paradigms used for cHL have been employed, with similar outcomes. Unlike cHL, late relapses may occur, as well as a propensity to transform to an aggressive B-cell non-Hodgkin lymphoma that underscores the importance of long-term follow-up and rebiopsy at the time of relapse. Deaths caused by NLPHL are uncommon, and in older series, secondary malignancies and other treatment-related toxicities contributed appreciably to overall mortality. Expression of CD20 in NLPHL has led to the evaluation of rituximab as a therapeutic option. Although results with single-agent rituximab in the front-line setting are inferior to conventional therapy, rituximab is a reasonable choice for relapsed disease because of the high overall response rate and excellent tolerability. Most patients have a long OS; therefore, overall goals of therapy should be to minimize the risk for relapse and long-term toxicity.

Nodular lymphocyte predominant Hodgkin lymphoma: a Lymphoma Study Association retrospective study

“…We cannot conclude from our data the possible impact of the absence of maintenance on our results, which are in line with the experience of the Stanford University and the British Columbia Cancer Agency. 23,24 However, the benefit of rituximab maintenance has not been confirmed in a recent series. 3 The international guidelines recommend an initial treatment with 6 or without rituximab alone, 5 and rituximab could be a reasonable choice at relapse.…”

“…3 The international guidelines recommend an initial treatment with 6 or without rituximab alone, 5 and rituximab could be a reasonable choice at relapse. 25 The management of our patients at first relapse/progression showing a low number of patients in the different treatment groups, and the heterogeneity of reported series in terms of features of disease and therapeutic options, 1,8,23 do not allow for a clear definition of the treatment of relapsed NLPHL. High-dose chemotherapy with ASCT was the salvage treatment for a few of our patients in the absence of documented histological transformation, and we cannot make a judgement on the possible benefit of ASCT in this setting.…”

Characteristics and Outcomes of Patients With Nodular Lymphocyte-Predominant Hodgkin Lymphoma Versus Those With Classical Hodgkin Lymphoma: A Population-Based Analysis

“…The declining use of radiation may stem in large part from concern for late effects as data concerning late effects of radiation in HL survivors have emerged over the past two and a half decades. Nonradiationbased strategies including surgical resection followed by observation and observation alone have been investigated, particularly in the pediatric population (21)(22)(23), but many of these patients develop relapse and observation alone is not routinely recommended, with the exception of certain pediatric patients (3). In the recent Children's Oncology Group protocol for pediatric patients with early-stage HL, patients are treated with upfront chemotherapy with no further treatment if a complete response was achieved (Combination Chemotherapy Followed by Radiation Therapy in Treating Young Patients With Newly Diagnosed Hodgkin's Lymphoma;…”

“…The clinical characteristics, treatment approach, and outcomes of patients with NLPHL differ from those of classic HL (CHL) (1)(2)(3)(4). NLPHL patients tend to present at an earlier stage and have an improved prognosis compared to patients with CHL (2,5).…”

CEPP regimen (cyclophosphamide, etoposide, procarbazine and prednisone) as initial treatment for Hodgkin lymphoma patients presenting with severe abnormal liver function

“…Hodgkin lymphoma is a highly curable disease in the current era. Chemotherapy with doxorubicin, bleomycin, vinblastine and dacarbazine (ABVD regimen) remains the mainstay of treatment [4,5,9,10]. As per American Cancer Society’s estimates for HL in the United States, the 5-year and 10-year survival rates reached 85% and 80%, respectively [11].…”