Nodular Lymphocyte-Predominant Hodgkin Lymphoma: Review of Current Literature and Case Discussion

Abstract

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare subset of Hodgkin lymphoma (HL). It has a distinct clinical and pathological presentation. Unlike classic HL, where the predominant malignant cells are Reed Sternberg cells, the malignant cells in NLPHL are known as lymphocyte predominant (LP) cells, with their own unique immunohistochemistry antigen expression and staining pattern. Based on risk stratification and staging of the disease, treatment can range from active surveillance in asymptomatic patients with no organ compromise or bulky disease, to aggressive chemotherapeutic agents in advanced disease. Guidelines on which of these chemotherapy regimens would offer the most benefit to our patients are limited due to lack of randomized-controlled studies. Majority of the current prospective data on treatment were inclusive of both HL and NLPHL. Thus, the regimens employed in treatment of NLPHL are similar to the ones used in HL, though NLPHL is often viewed as its own distinct entity. This article aims to review the current literature and future advances on treatment of this rare disease.

Nodular lymphocyte predominant Hodgkin lymphoma with T-cell/histiocyte - rich large B-cell lymphoma pathohistological characteristics and extranodal presentation: Case report and literature review

“…There is a correlation between the pathohistological type of NLPHL and its clinical presentation. In cases of NLPHL that have histopathological resemblance with THRLBCL, a more aggressive clinical presentation, including liver and spleen infiltration, can be expected, unlike the indolent clinical course that is common for other types of NL-PHL [1,[11][12][13]. Most of the patients with NLPHL, 80% of them, have limited stage of the disease.…”

“…There are different treatment approaches in patients with NLPHL depending on pathohistological type, clinical presentation and stage of the disease. Treatment may include active surveillance, radiation therapy, immunotherapy or chemotherapy [1,11,14]. Variant histology patterns, A, B and C or Grade 1, and D, E and F or Grade 2, along with clinical features, should be considered while deciding on the therapy course.…”

“…In advanced stages, the therapy choice is a more ag-gressive approach, including BEACOPPesc. protocol (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisone), or administration of the R-CHOP protocol [1,11,14,17,18]. A report from The German Hodgkin Study Group (GHSG) in 2013. that included 413 cases, studied differences between typical and variant histopathological patterns and their prognostic impact.…”

“…In some patients, despite the indolent character of the NLPHL and administration of therapy, progression or relapse of the disease occurs [1,11,14,16]. According to a retrospective analysis of GHSG, the prognosis for patients with NLPHL is at least as good as that for patients with cHL, but the rate of relapse is higher [1,13,16,[19][20][21].…”

“…In the previously mentioned study, one patient with NLPHL and THRLBCL that was treated with R-CHOP protocol, had progression within 1 year. (18) In NLPHL the incidence of transformation into DLBCL is higher than in HL [11,22,23]. Transformation is more likely in patients with advanced stage, with spleen involvement, despite aggressive treatment [22,24].…”