nodular lymphocyte predominant hodgkin lymphoma

DIAGNOSIS AND STAGING OF HODGKIN LYMPHOMA. Hodgkin lymphoma (HL) is composed of two distinct disease entities: classical HL (cHL) and nodular lymphocyte predominant HL (NLPHL). NLPHL is a rare entity with a specific therapeutic management. The subgroups of cHL are nodular sclerosis, mixed cellularity, lymphocyte depletion and lymphocyte-rich HL. The initial diagnosis of HL is made by a biopsy to analyze with accuracy the architecture of the lymph node. Most HL patients present with supradiaphragmatic lymphadenopathy...

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare B cell-derived lymphoma entity accounting for ≈5% of all Hodgkin lymphoma (HL) cases. In recent decades, patients with newly diagnosed NLPHL have usually been treated very similarly to classical HL (cHL). The 10-year overall survival rates with HL-directed approaches are in excess of 90%. However, pathological and clinical characteristics of NLPHL resemble indolent B-cell non-Hodgkin lymphoma (B-NHL) in some aspects. Thus, nodular lymphocyte-predominant B-cell lymphoma has been proposed as an alternative name, and the use of B-NHL-directed treatment strategies has become more common in NLPHL despite limited data...

Hodgkin's lymphoma (HL) is a malignancy that is typically B-cell in origin. HL can be further classified into classical HL and nodular lymphocyte-predominant HL (NLPHL). NLPHL is a rare lymphoma. It commonly presents locally with palpable firm lymphadenopathy or mediastinal mass seen on chest imaging. Some patients may have B symptoms (fever, night sweats, and unintentional weight loss), splenomegaly, and hepatomegaly. We describe a case of NLPHL in a 32-year-old male with classical findings of this rare class of HL...

BACKGROUND: Hematological malignancies are an infrequent but important cause of liver dysfunction. There are several mechanisms by which this can occur, including direct malignant infiltration of the hepatic parenchyma and/or vasculature, vanishing bile duct syndrome, and paraneoplastic hepatitis. Paraneoplastic hepatitis is an extremely rare mechanism by which a hematological malignancy can cause liver dysfunction, and we present the first case, to our knowledge, of paraneoplastic hepatitis caused by nodular lymphocyte-predominant Hodgkin lymphoma in the literature...

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Since the approval of brentuximab vedotin (BV), assessment of CD30 status by immunohistochemistry gained increasing importance in the clinical management of patients diagnosed with CD30-expressing lymphomas, including classical Hodgkin lymphoma (CHL). Paradoxically, patients with low or no CD30 expression respond to BV. This discrepancy may be due to lack of standardization in CD30 staining methods. In this study, we examined 29 cases of CHL and 4 cases of nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) for CD30 expression using a staining protocol that was designed to detect low CD30 expression levels, and an evaluation system similar to the Allred scoring system used for breast cancer evaluation...

INTRODUCTION: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare lymphoma entity accounting for roughly 5% of all Hodgkin lymphoma (HL) cases. In contrast to classical HL, the malignant cells in NLPHL are positive for CD20 but lack CD30. The disease usually has an indolent clinical course resulting in high long-term survival rates. AREAS COVERED: In this review, treatment options for NLPHL are summarized and factors that may help to individualize treatment are discussed...

The optimal first-line treatment for nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) diagnosed in early stages is largely undefined. We, therefore, analyzed 100 NLPHL patients treated in the randomized HD16 (early-stage favorable; n = 85) and HD17 (early-stage unfavorable; n = 15) studies. These studies investigated the omission of consolidation radiotherapy (RT) in patients with a negative interim positron emission tomography (iPET) (ie, Deauville score <3) after chemotherapy (HD16: 2× doxorubicin, bleomycin, vinblastine, and dacarbazine [ABVD]; HD17: 2× escalated bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone [BEACOPP] plus 2× ABVD)...

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Therapy results in pediatric Hodgkin lymphoma reflect remarkable progress in pediatric oncology. In the last decade, relevant development of new therapeutic options for children with refractory or relapsed disease has been made. In this study, we retrospectively analyzed therapy results and risk factors in children treated in a single oncology center according to five therapeutic protocols. Data from 114 children treated by a single institution between 1997 and 2022 were analyzed. Classic Hodgkin lymphoma therapy results were divided into four therapeutic periods: 1997-2009, 2009-2014, 2014-2019, and 2019-2022...

BACKGROUND: Studies are needed to assess risk factors pertinent to the incidence of secondary malignancies among childhood and adolescent lymphoma survivors. We aimed to identify risk factors pertinent to the incidence of secondary malignancies and subsequently establish a clinically practical predictive nomogram. METHODS: A total of 5561 patients who were diagnosed with primary lymphoma below the age of 20 years between 1975 and 2013 and survived for at least 5 years were identified...

Hodgkin lymphoma is a B-cell neoplasm that typically presents with localized, nodal disease. Tissues are characterized by few large neoplastic cells, usually comprising less than 10% of tissue cellularity, present in a background of abundant nonneoplastic inflammatory cells. This inflammatory microenvironment, although key to the pathogenesis, can make diagnosis a challenge because reactive conditions, lymphoproliferative diseases, and other lymphoid neoplasms may mimic Hodgkin lymphoma and vice versa. This review provides an overview of the classification of Hodgkin lymphoma, its differential diagnosis, including emerging and recently recognized entities, and strategies to resolve challenging dilemmas and avoid diagnostic pitfalls...

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a Hodgkin lymphoma expressing functional B-cell receptors (BCRs). Recently, we described a dual stimulation model of IgD+ lymphocyte-predominant (LP) cells by Moraxella catarrhalis antigen RpoC and its superantigen MID/hag, associated with extralong CDR3s and HLA-DRB1*04 or HLA-DRB1*07 haplotype. Aim of the present study was to extend the antigen screening to further bacteria and viruses. Fabs of 7 new and 15 previously reported cases were analyzed...

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare variant of Hodgkin lymphoma characterized by a persistent risk of relapse but an excellent overall survival. Historically, it was treated similarly to classic Hodgkin lymphoma, but efforts have been made to deintensify treatment due to risk of late toxicity associated with intensive therapy. For patients with completely resected stage IA NLPHL, no further treatment may be considered, particularly for pediatric patients. For those with stage I-II NLPHL without risk factors such as B symptoms, sites>2, or variant pattern histology, lower intensity treatment with radiotherapy or chemotherapy alone may be sufficient...

Nodular Lymphocyte predominant Hodgkin lymphoma (NLPHL) are rare lymphomas in pediatric patients comprising less than 10 % of all Hodgkin lymphoma (HL). They are for the most part diagnosed at stage I or II and indolent with lymphadenopathy often preceding the diagnosis by many months/years. Survival is excellent. Historically, patients were treated according to classical HL protocols. Due to high toxicity and excellent prognosis, management of NLPHL shifted to de-escalation protocol with good results...

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare entity whose neoplastic cells retain a B-cell phenotype with expression of CD20. Radiotherapy is recommended for favorable stage IA disease while for other stages guidelines suggest therapeutic strategies similar to those used for classic HL. The role of rituximab, although quite widespread, is not completely elucidated. We retrospectively analyzed baseline characteristics of 308 consecutive patients with NLPHL diagnosed in 19 Italian centers from 2000 to 2018...

We leveraged population-based clinical and healthcare data to identify treatment patterns and long-term outcomes among adolescents and young adults (AYA) with nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL). All Ontario, Canada, AYA aged 15-21 years at diagnosis with NLPHL between 1992 and 2012 were identified, and their detailed clinical data were collected. Linkage to healthcare databases identified additional events (subsequent malignant neoplasms [SMN], relapses and deaths). Event-free survival (EFS) and overall survival (OS) were compared by locus of care (adult vs...

Hodgkin lymphoma (HL) is a rare type of lymphoma with unique histologic, immunophenotypic, and clinical features. It represents approximately one-tenth of lymphomas diagnosed in the United States and consists of two subtypes: classical Hodgkin's lymphoma (cHL), which accounts for majority of HL cases, and nodular lymphocyte predominant Hodgkin lymphoma represent approximately 5% of Hodgkin lymphoma cases. From this point, we will be focusing on cHL in this review. In general, it is considered a highly curable disease with first-line chemotherapy with or without the addition of radiotherapy...

Two new classifications were recently released: the 5th edition of the WHO classification of hematolymphoid tumors and the International Consensus Classification (ICC) drafted by the Clinical Advisory Committee. In the preparation of both classifications, the previously existing lymphoma categories were reevaluated according to recently obtained data on clinical, morphological, and molecular findings. In this review we summarize the current placements of classic and nodular lymphocyte predominant Hodgkin lymphoma and their relevant differential diagnoses...

While cervical lymphadenopathy is common in children, a decision model for detecting high-grade lymphoma is lacking. Previously reported individual lymphoma-predicting factors and multivariate models were not sufficiently discriminative for clinical application. To develop a diagnostic scoring tool, we collected data from all children with cervical lymphadenopathy referred to our national pediatric oncology center within 30 months ( n = 182). Thirty-nine putative lymphoma-predictive factors were investigated...