High Familial Risk in Nodular Lymphocyte-Predominant Hodgkin Lymphoma

Saarinen, Silva; Pukkala, Eero; Vahteristo, Pia; Mäkinen, Markus J.; Franssila, Kaarle; Aaltonen, Lauri A.

doi:10.1200/jco.2012.43.5958

Cited by 51 publications

(35 citation statements)

References 18 publications

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“…The results of the current study suggest that susceptibility to NLPHL may be mediated by interacting factors related to race and sex. The sex‐specific component is corroborated by the Finnish registry study of first‐degree relatives of patients with NLPHL, among whom female relatives were found to have twice as high an incidence of NLPHL as male relatives . Identifying the molecular basis of NLPHL predisposition among African American individuals will require further dedicated research.…”

Section: Discussionmentioning

confidence: 93%

Race‐specific features and outcomes of nodular lymphocyte‐predominant Hodgkin lymphoma: Analysis of the National Cancer Data Base

Olszewski

Shrestha

Cook

2015

Cancer

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BACKGROUND The incidence of nodular lymphocyte‐predominant Hodgkin lymphoma (NLPHL) is higher among African Americans than among other races, but to the authors' knowledge, the characteristics of NLPHL in this population have not been evaluated. The authors compared clinical features, treatments, and survival of black and white patients with NLPHL using the National Cancer Data Base. METHODS The authors extracted the records of 602 black and 1950 white patients with NLPHL who were diagnosed between 1998 and 2011. Overall survival (OS) was compared using the log‐rank test. RESULTS Black patients were on average younger than white patients (median age, 42 years vs 45 years; P =.0001), more often female (49% vs 29%; P<.0001), and more likely to have the axillary lymph nodes as the primary disease site (25% vs 17%; P =.0002). They also had unfavorable socioeconomic characteristics, a higher rate of no treatment in patients with early‐stage disease, and a longer time to therapy initiation (median, 53.5 days vs 47 days; P<.0001). Despite this, the authors found no significant difference between the races with regard to stage distribution or survival (P =.39). OS at 7 years was 90.1% in patients with early‐stage (American Joint Committee on Cancer stage IA/B, IIA) and 79.4% in patients with advanced stage (American Joint Committee on Cancer stage IIB, III/IV) NLPHL. Survival in the early stage of disease was not found to be significantly different after various treatment strategies (stratified log‐rank P = .18), except that the administration of chemotherapy was associated with a better outcome in black patients (log‐rank P =.011 vs P =.81 for white patients). CONCLUSIONS Differences in clinical presentation suggest the interaction of race‐specific and sex‐specific susceptibility factors for NLPHL. Further research is needed to elucidate these factors, and to investigate possible heterogeneous effects of treatments by race. Clinical trials comparing standard treatment strategies are unlikely to detect differences in OS among patients with early‐stage NLPHL. Cancer 2015;121:3435–43. © 2015 American Cancer Society.

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Section: Discussionmentioning

confidence: 93%

Race‐specific features and outcomes of nodular lymphocyte‐predominant Hodgkin lymphoma: Analysis of the National Cancer Data Base

Olszewski

Shrestha

Cook

2015

Cancer

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“…1 In a Finnish registry, the standardized incidence ratio for 18 Asymptomatic adenopathy can precede diagnosis by months to years. The largest series is a retrospective analysis by the German Hodgkin Study Group (GHSG) in which 394 patients with NLPHL were compared with 7904 patients with cHL.…”

Section: Clinical Featuresmentioning

confidence: 99%

How I treat nodular lymphocyte predominant Hodgkin lymphoma

Advani¹,

Hoppe

2013

Blood

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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.

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“…The median age at initial diagnosis is ;40 years. 2,3 In contrast to classical HL (cHL), the majority of NLPHL patients are diagnosed in the early favorable stages. The long-term remission rate among these patients exceeds 90% when they are treated with current standard approaches.…”

Section: Introductionmentioning

confidence: 99%

The prognostic impact of variant histology in nodular lymphocyte-predominant Hodgkin lymphoma: a report from the German Hodgkin Study Group (GHSG)

Hartmann

Eichenauer

Plütschow

et al. 2013

Blood

173

144

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Key Points• Histopathologic variants of nodular lymphocytepredominant Hodgkin lymphoma are associated with advanced stage and increased relapse rate.• A prognostic score combining histopathologic and clinical features can allocate patients to 3 defined risk groups.Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) accounts for approximately 5% of all Hodgkin lymphoma cases. The aim of this study was to evaluate the prognostic implication of histopathologic NLPHL variants. Biopsies of 423 NLPHL patients treated within 9 prospective clinical trials performed by the German Hodgkin Study Group were classified as tumor cell-rich cases (n 5 10), typical NLPHL (n 5 308), or histopathologic variants (n 5 105). Histopathologic variants were characterized by the presence of lymphoma cells outside the B-cell nodules or B-cell depletion of the microenvironment. Compared with typical NLPHL, histopathologic variants were associated with advanced disease (29.5% vs 14.6%, P 5 .0012) and a higher relapse rate (18.1% vs 6.5% at 5 years, P 5 .0009). Variant histology represented an independent prognostic factor (odds ratio 5 2.955) in a multivariate model of progression/relapse. A prognostic score, including the risk factors variant histopathologic growth pattern, low serum albumin, and male gender, was derived from this model and allowed the definition of 3 distinct risk groups. NLPHL patients presenting with histopathologic variants have a poorer outcome compared with those showing typical histology. The newly developed prognostic score combining histologic and clinical features allows allocating NLPHL patients to defined risk groups. (Blood. 2013;122(26):4246-4252) Continuing Medical Education online This activity has been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education through the joint sponsorship of Medscape, LLC and the American Society of Hematology. Medscape, LLC is accredited by the ACCME to provide continuing medical education for physicians. Medscape, LLC designates this Journal-based CME activity for a maximum of 1.0 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity. All other clinicians completing this activity will be issued a certificate of participation. To participate in this journal CME activity: (1) review the learning objectives and author disclosures; (2) study the education content; (3) take the post-test with a 70% minimum passing score and complete the evaluation at http://www.medscape.org/journal/blood; and (4) view/print certificate. For CME questions, see page 4292. There is an Inside Blood commentary on this article in this issue.The publication costs of this article were defrayed in part by page charge payment. Therefore, and solely to indicate this fact, this article is hereby marked "advertisement" in accordance with 18 USC section 1734. 1 NLPHL more commonly affects males (male-female ratio, 3:1). The median age at...

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High Familial Risk in Nodular Lymphocyte-Predominant Hodgkin Lymphoma

Abstract: Our results implicate an unexpectedly high familial component in the development of NLPHL. Research is warranted to identify the putative genetic and environmental factors underlying this finding and to develop strategies for better management of patients with NLPHL and their relatives.

Cited by 51 publications

References 18 publications

Race‐specific features and outcomes of nodular lymphocyte‐predominant Hodgkin lymphoma: Analysis of the National Cancer Data Base

Race‐specific features and outcomes of nodular lymphocyte‐predominant Hodgkin lymphoma: Analysis of the National Cancer Data Base

How I treat nodular lymphocyte predominant Hodgkin lymphoma

The prognostic impact of variant histology in nodular lymphocyte-predominant Hodgkin lymphoma: a report from the German Hodgkin Study Group (GHSG)

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