Nodular Lymphocyte Predominant Hodgkin Lymphoma Research Articles

Relapsed nodular lymphocyte-predominant Hodgkin lymphoma presenting as severe paraneoplastic hepatitis: a case report

BackgroundHematological 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.Case presentationA 28-year-old Caucasian male presented with 3 weeks of fatigue, epigastric pain, and jaundice. His medical history was significant for early stage nodular lymphocyte-predominant Hodgkin lymphoma in the cervical region in remission for 5 years after primary treatment with involved-field radiotherapy. Liver biochemistry was normal at the time of treatment for lymphoma and there was no known liver disease before the current presentation. On physical examination, there was scleral icterus and ecchymoses, but no evidence of hepatic encephalopathy, other stigmata of chronic liver disease, or lymphadenopathy. A computed tomography scan of his neck, chest, abdomen, and pelvis showed heterogeneous enhancement of the liver, multiple enlarged upper abdominal lymph nodes, and an enlarged spleen with multiple rounded lesions. Portal and hepatic veins were patent. Initial workup for viral, autoimmune-, toxin-, and medication-related hepatitis was negative. A transjugular liver biopsy was performed with histology showing a predominantly T-cell mediated hepatitis with very extensive multiacinar hepatic necrosis, but no evidence of lymphoma within the liver. Retroperitoneal lymph node biopsy revealed nodular lymphocyte-predominant Hodgkin lymphoma. The patient’s symptoms, bilirubin, and transaminases improved significantly after treatment with oral prednisolone and a staged introduction of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy.ConclusionsNodular lymphocyte-predominant Hodgkin lymphoma may cause paraneoplastic hepatitis. Physicians should be aware of the possibility of this life-threatening presentation and the importance of early liver biopsy and treatment before acute liver failure occurs. Interestingly, paraneoplastic hepatitis did not occur when nodular lymphocyte-predominant Hodgkin lymphoma was first diagnosed and confined to the cervical region, but was the presenting feature of the recurrence below the diaphragm.

Treatment of Nodular Lymphocyte-Predominant Hodgkin Lymphoma: Where Do We Stand? Where Do We Go?

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. Given the often indolent clinical course of NLPHL, even in the case of relapse, the majority of patients with disease recurrence do not require high-dose chemotherapy and autologous stem cell transplantation but are treated sufficiently with low-intensity approaches such as single-agent anti-CD20 antibody treatment. The establishment of novel prognostic scores for NLPHL patients may optimize risk group and treatment allocation in newly diagnosed and relapsed disease.

Interim PET-guided treatment for early-stage NLPHL: a subgroup analysis of the randomized GHSG HD16 and HD17 studies

AbstractThe 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). Patients with NLPHL treated in the HD16 and HD17 studies had 5-year progression-free survival (PFS) rates of 90.3% and 92.9%, respectively. Thus, the 5-year PFS did not differ significantly from that of patients with classical Hodgkin lymphoma treated within the same studies (HD16: P = .88; HD17: P = .50). Patients with early-stage favorable NLPHL who had a negative iPET after 2× ABVD and did not undergo consolidation RT tended to have a worse 5-year PFS than patients with a negative iPET who received consolidation RT (83% vs 100%; P = .05). There were 10 cases of NLPHL recurrence. However, no NLPHL patient died during follow-up. Hence, the 5-year overall survival rate was 100%. Taken together, contemporary Hodgkin lymphoma-directed treatment approaches result in excellent outcomes for patients with newly diagnosed early-stage NLPHL and, thus, represent valid treatment options. In early-stage favorable NLPHL, consolidation RT appears necessary after 2× ABVD to achieve the optimal disease control irrespective of the iPET result.

Do Hispanic individuals have similar overall survival of nodular lymphocyte-predominant Hodgkin’s lymphoma? A national analysis.

e19512 Background: Nodular lymphocyte-predominant Hodgkin’s Lymphoma (NLPHL) is a rare subtype of Hodgkin’s Lymphoma (HL) accounting for approximately 5% of all cases of HL in the United States (US). (PMID: 29763144 ) The prognosis overall for NLPHL is exceptionally good. (PMID: 34923571) Much of the clinical outcome data for NLPHL is from European countries or the US without racial sub-group analysis available. (PMID: 34923571, PMID: 33976110, PMID: 29222274 ) Few studies examine if there are disparities in outcomes nationally within each HL subtype when comparing Hispanics and non-Hispanics. (PMID: 22241896, PMID: 26198444) This study looks at the impact of ethnicity across several key variables and the survival outcomes of this cancer in Hispanics (HI) compared to Non-Hispanics (NH) within the US, using Surveillance, Epidemiology, and End Results (SEER) database. Methods: Data was analyzed on NLPHL patients in the US and reported to the SEER database between 2000 -2018. Patient demographics, survival, and clinical course were evaluated with Kaplan-Meier, Cox regression, multivariate analysis, and propensity score matching. Results: 2,532 of NLPHL patients, 11% were HI, and 89% were NH. &gt; 65% of those diagnosed were male in both groups. HI pts were younger, with a median age of 35 years old vs 45 years old (p &lt; 0.001); the majority of HI (55%) were diagnosed in the age bracket of &lt; 40 years old vs the majority of NH 42% were diagnosed in the age bracket of &lt; 40 years old (p = &lt; 0.001) Both groups had a majority of diagnosis during the years 2015-2018 (p = 9.18). Regarding race, most of HI and NH were whites 89% vs 68%, followed by blacks 6% vs 26%. Both groups were most commonly Stage at the time of diagnosis ~30% (0.584). In terms of radiation therapy rates were similar ~37% (p = 0.287) On survival analysis, the survival probability at 2, 5, and 10 years of HI vs NH was 93% vs 96%, 89% vs 92%, and 84% vs 80%, respectively. The median survival time was not reached years for HI and was also not reached for NH, and there was no overall survival difference favoring HI/NH (p = 0.43) On multivariate analysis, when adjusting for age, those who were &gt; 80 years old and between 60-80 years old had worse OS compared to those &lt; 60 years old with HR 33.5 (CI 95% 20.6 – 54.5) and 4.3 (CI 95% 2.7 – 6.9). Regarding stage, stage III/IV had worse OS than stage I/II with HR 1.9 (95% CI 1.1 – 3.4) vs HR 2.4 (95% CI 1.0 - 5.6). Conclusions: Overall HI were younger at the time of diagnosis without a significant difference in stage of diagnosis. Median survival was not reached in both NH and HI which reflects the good prognosis associated with NHLPL. Despite HI being having a younger median age of diagnosis than NH this did not correlate with improved OS in HI vs NH despite younger showing improved OS. Given there are little few other studies examining HI vs NH outcomes in NLPHL more research is needed to evaluate possible confounders skewing these results.

17th International Conference on Malignant Lymphoma, Palazzo dei Congressi, Lugano, Switzerland, 13 - 17 June, 2023.

Introduction: THRLBCL is an orphan entity of large B-cell lymphomas (LBCL). It is dominated by stromal and immune components—especially T-cells and histiocytes—with similarities to nodular lymphocyte-predominant Hodgkin lymphoma. This microenvironment can produce immunomodulating molecules with therapeutic implications since recent reports suggest THRLBCL resistance to chimeric antigen receptor T-cells (CART). As CART-based therapies are challenging the role of autoHCT as second line consolidation for relapsed/refractory LBCL, we aimed to study the efficacy of autoHCT in THRLBCL in comparison with diffuse LBCL not other specified (DLBCL). Methods: Eligible for this EBMT registry analysis were adult patients who underwent a first autoHCT for THRLBCL between 2016 and 2020. These were compared to adult patients with DLBCL who received a first autoHCT during the same time period. Statistical analysis was descriptive and employed univariate and multivariate comparisons for the impact of baseline characteristics on survival endpoints. A 1:2 matching comparison was performed to better adjust for baseline differences between THRLBCL and DLBCL, respectively. Results: In total 11,151 patients (10,831 DLBCL, 320 THRLBCL) were identified. THRLBCL patients were younger (52 vs. 58 years), predominantly male (76% vs. 59%), had better performance status (ECOG 0: 97% vs. 92%) and less comorbidity (HCT-CI 0: 78% vs. 68%), and had a longer time from diagnosis to transplant (13 vs. 11 months) than DLBCL patients. In contrast, there were no significant differences regarding the proportion of patients receiving autoHCT during second-line (2L) treatment (87% vs. 82%) and sensitive disease status at autoHCT (95% vs. 92%) between THRLBCL and DLBCL, respectively. On logrank comparisons including all patients, THRLBCL was associated with a better 2-year overall survival (OS; 79% vs. 73%; p = 0.05) and progression-free survival (PFS; 72% vs. 61%) than DLBCL. However, when comparing 139 THRLBCL with 278 DLBCL patients matched for age, sex, disease status, performance status (PS), comorbidities (HCT-CI), and time from diagnosis, the outcome differences were no longer significant. Multivariate analyses adjusting for age, gender, disease status, PS, HCT-CI, and time from diagnosis identified higher age, refractory disease, >12 months from diagnosis, and poorer PS as significant risk factors for reduced OS and PFS. Of note, diagnosis THRLBCL was an independent predictor of reduced relapse risk (HR 0.73; p = 0.04). Conclusions: AutoHCT is an effective salvage therapy for relapsed/refractory THRLBCL sensitive to salvage therapy. Superior outcome of autoHCT for THRLBCL compared to DLBCL may be partly explained by more favorable baseline features of the THRLBCL group in terms of age, and PS. The 72% PFS-benchmark shown for autoHCT here has to be considered when deciding about 2L treatment strategies for THRLBCL. Keywords: Aggressive B-cell non-Hodgkin lymphoma, Stem Cell Transplant Conflicts of interests pertinent to the abstract. S. Renders Honoraria: Gilead P. Dreger Honoraria: Novartis, Kite, BMS, Miltenyi

Integrative approach to diagnosis and subsequent successful therapy of refractory follicular lymphoma using obinutuzumab in combination with bendamustine. Clinical observation

In the presented observation, a female patient with diagnosis of nodular lymphocyte-predominant Hodgkin lymphoma (advanced stages) received R-ABVD regimen therapy. Return of clinical symptoms observed at the onset of the disease, as well as renewed growth of the lymph nodes after 4th therapy cycle showed refractory disease. Considering the results of earlier studies, generally showing high effectiveness of primary treatment of patients with nodular lymphocyte-predominant Hodgkin lymphoma, reevaluation of immunomorphological type of lymphoproliferative disease was performed. After change of diagnosis to grade 3A follicular lymphoma, therapy was changed to obinutuzumab in combination with bendamustine leading to fast positive antitumor effect.

Therapy results in pediatric Hodgkin lymphoma — does less mean better? Experience from a single children’s oncology center

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. For nodular lymphocyte-predominant Hodgkin lymphoma, data from one therapeutic protocol was analyzed. For the entire group, the 5-year probability of overall survival was 93.5%. There were no statistically significant differences between therapeutic periods. The occurrence of B symptoms at diagnosis and incidence of relapse were risk factors for death (p = 0.018 and p < 0.001). Relapse occurred in 5 cases. The 5-year probability of relapse-free survival for the entire group was 95.2%, without significant differences between groups. Patients treated between 1997 and 2009 had over a sixfold higher risk for events, defined as primary progression, relapse, death, or incidence of secondary malignancies (OR = 6.25, p = 0.086). The 5-year probability of event-free survival for all patients was 91.3%. Five patients died, and the most common cause of death was relapse. Modern therapeutic protocols in pediatric Hodgkin lymphoma are marked by excellent outcomes. Patients with disease relapses have a notably high risk of death, and the development of new therapeutic options for this group remains one of the main goals of current trials.

Long-Term Risk of Subsequent Malignant Neoplasms Among Childhood and Adolescent Lymphoma Survivors (1975-2013): A Population-Based Predictive Nomogram.

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. 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. Standardized incidence ratio (SIR) and excess risk (ER) analysis were performed by sex, age, and year when primary lymphoma was diagnosed, sites and types of primary lymphoma, and therapy strategies. Univariable and multivariable logistic regression were used to identify independent risk factors for adolescent and childhood lymphoma-related secondary malignancies. Based on 5 factors (age, time from lymphoma diagnosis, gender, lymphoma type, and therapy), a nomogram for predicting the risk of a secondary malignancy for patients with childhood and adolescent primary lymphoma was established. Among 5561 lymphoma survivors, 424 developed a secondary malignancy. Females (SIR = 5.34, 95% CI, 4.73-5.99; ER = 50.58) exhibited a higher SIR and ER than males (SIR = 3.28, 95% CI, 2.76-3.87; ER = 15.53). Blacks were at a higher risk than Caucasians or others. Nodular lymphocyte-predominant Hodgkin lymphoma survivors exhibited typically high SIR (13.13, 95% CI, 6-24.92) and ER (54.79) among all lymphoma classifications. Lymphoma survivors who underwent radiotherapy, whether they received chemotherapy or not, had typically higher SIR and ER. Among all types of secondary malignancies, "bone and joint neoplasms" (SIR = 11.07, 95% CI, 5.52-19.81) and "soft tissue neoplasms" (SIR = 12.27, 95% CI, 7.59-18.76) presented significantly high SIR whereas "breast cancer" and "endocrine cancer" associated with higher ER. The median diagnosis age of secondary malignancies was 36 years old, and the median time interval between the diagnosis of two malignancies was 23 years. A nomogram was constructed to predict the risk of secondary malignancies in patients diagnosed with primary lymphoma before 20 years of age. After internal validation, the AUC and C-index of the nomogram are 0.804 and 0.804, respectively. The established nomogram provides a convenient and reliable tool for predicting the risk of a secondary malignancy among childhood and adolescent lymphoma survivors, concluding significant concern for lymphoma survivors with high-risk estimates.

B-cell receptor reactivity against Rothia mucilaginosa in nodular lymphocyte-predominant Hodgkin lymphoma.

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a Hodgkin lymphoma expressing functional B-cell receptors (BCR). Recently, we described a dual stimulation model of IgD+ lymphocyte-predominant cells by Moraxella catarrhalis antigen RpoC and its superantigen MID/hag, associated with extralong CDR3 and HLA-DRB1*04 or HLADRB1* 07 haplotype. The aim of the present study was to extend the antigen screening to further bacteria and viruses. The fragment antibody-binding (Fab) regions of seven new and 15 previously reported cases were analyzed. The reactivity of non-Moraxella spp.-reactive Fab regions against lysates of Rothia mucilaginosa was observed in 5/22 (22.7%) cases. Galactofuranosyl transferase (Gltf) and 2,3-butanediol dehydrogenase (Bdh) of R. mucilaginosa were identified by comparative silver- and immuno-staining in two-dimensional gels, with subsequent mass spectrometry and validation by western blots and enzyme-linked immunosorbent assay. Both R. mucilaginosa Gltf and Bdh induced BCR pathway activation and proliferation in vitro. Apoptosis was induced by recombinant Gltf/ETA'-immunotoxin conjugates in DEV cells expressing recombinant R. mucilaginosa-reactive BCR. Reactivity against M. catarrhalis RpoC was confirmed in 3/7 newly expressed BCR (total 10/22 reactive to Moraxella spp.), resulting in 15/22 (68.2%) cases with BCR reactivity against defined bacterial antigens. These findings strengthen the hypothesis of bacterial trigger contributing to subsets of NLPHL.

Recommandations de la SFCE pour la prise en charge du lymphome de Hodgkin nodulaire à prédominance lymphocytaire de l’enfant et de l’adolescent

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. No treatment beyond surgical resection was proposed for localized unique nodal disease completely resected. The closed European protocol (EuroNet PHL LP1) evaluated the efficacy of low intensity chemotherapy protocol based on CVP courses (cyclophosphamide vinblastine prednisone) for stage IA/IIA not fully resected. Final results are not yet available. Advanced stage NLPHL are rare and there is no clinical trial and no consensus treatment in children. The SFCE lymphoma committee recently established recommendations for staging and treatment of limited and advanced NLPHL in children based on current practices and published results. The goal was to allow homogeneous practice on a national scale. If incomplete resection for patients with stage I/IIA combination of low intensity chemotherapy (CVP) and rituximab is recommended. For intermediary and advanced stage intensification with AVD (adriamycine vinblastine dacarbazine) or CHOP courses (cyclophosphamide doxorubicine vincristine prednisone) combined with rituximab are advocated. In children, there is no indication for first-line local treatment with radiotherapy.

MEF2B is the ideal immunohistochemical marker to highlight neoplastic LP cells in nodular lymphocyte-predominant Hodgkin lymphoma.

MEF2B is the ideal immunohistochemical marker to highlight neoplastic LP cells in nodular lymphocyte-predominant Hodgkin lymphoma.

Treatment patterns and outcomes in adolescents and young adults with nodular lymphocyte-predominant Hodgkin lymphoma: an IMPACT cohort study.

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. paediatric) and predictors of outcomes determined. Of 1014 AYA with Hodgkin lymphoma, 54 (5.3%) had NLPHL; 15 (27.8%) were treated at a paediatric centre. No paediatric centre patient received radiation only versus 16 (41.0%) of adult centre patients. Excision only was more common in paediatric centres (p < 0.001). The 20-year EFS and OS rates were 82.9% ± 5.2% and 100% respectively. Advanced stage (hazard ratio: 4.9, 95% CI: 1.3-18.4; p=0.02) was associated with inferior EFS. Although the 25-year cumulative incidence of SMN was 19.3% ± 9.6% for the entire cohort, there were no SMN among the patients treated with excision only. AYA with NLPHL have outstanding long-term survival. Resection alone was rare outside of paediatric institutions but associated with excellent outcomes. Given substantial SMN risks, chemotherapy-sparing and radiation-sparing strategies for appropriate subsets of patients are warranted.

Role of Rituximab Addition to First-line Chemotherapy Regimens in Nodular Lymphocyte-predominant Hodgkin Lymphoma: A Study by Fondazione Italiana Linfomi.

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. With a median follow-up of 8.4 years (interquartile range: 4.5-12.4) for treated patients, median overall survival (OS) was not reached and estimated 5-year OS was 97.8% and 5-year progression-free survival (PFS) was 84.5%. Five-year cumulative incidence of histological transformation was 1.4%, 95% confidence interval (CI), 0.5%-3.8%. After adjusting for lymphocyte count, splenic involvement, bulky disease and B symptoms (fever, drenching night sweats, unintentional loss >10% of body weight within the preceding 6 months), patients with stage II or more showed superior PFS with immunochemotherapy in comparison to chemotherapy alone (hazard ratio = 0.4, 95% CI, 0.2-0.8; P = 0.015). Our data suggest an advantage of the use of rituximab combined with chemotherapy ± radiotherapy in the treatment of stage II-III-IV NLPHL.

Pediatric Lymphoma Incidence at Children's Hospital Benghazi from 2014 to 2023

Background: Malignant lymphoma is caused by the neoplastic growth of lymphocytes and can manifest itself diffusely or in one or more lymph node. Hodgkin lymphoma and non-Hodgkin lymphoma commonly arise from lymphoid tissue. Hodgkin lymphoma is rare B-cell malignant neoplasm it is mainly divided into two main subtype: classical Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin Lymphoma. Non-Hodgkin lymphoma is malignant disorder arising from cell of the immune system and typically present as lymphadenopathy or solid tumor. Objective: Aim of this research is detection of Pediatric lymphoma prevalence and the most common group affected in both age and gender in Children’s Hospital - Benghazi. Methods: Cross sectional study was conducted on 59 cases of pediatric lymphoma. The cases were admitted to Children’s Hospital - Benghazi. within the time period from January 2014 to May 2023. Data were distributed on the basis of age, gender, year of diagnosis, histological type, and others. Result: The highest number recorded was 9 cases in the years 2017, 2019 and 2020 and the lowest number of cases were at the year 2018 with only 2 cases. The average age of the cases found in the archive was 7.7 year of age. The gender distribution was 41 males, in comparison with only 18 females. The most common type of lymphoma would be Hodgkin lymphoma with 34 cases, histological reports showed the most common subtype of Non-Hodgkin lymphoma was Burkitt lymphoma with 9 cases. Conclusion: Pediatric lymphoma in Children’s Hospital Benghazi had a clinical presentation in favor of Hodgkin lymphoma .in this research it was detected the distribution of males was more predominant than females. Most cases found were at 3-4 years of age. medical records showed Stage 3 was the most common stage of the tumor.

Classification of Hodgkin lymphoma and related entities : News and open questions

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.

Advances in the treatment of Hodgkin lymphoma: Current and future approaches.

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. However, there are patients with disease that relapses or fails to respond to frontline regimens and the standard treatment modality for chemo sensitive cHL is high dose chemotherapy followed by autologous hematopoietic stem cell transplant (AHSCT). In recent years, targeted immunotherapy has revolutionized the treatment of cHL while many novel agents are being explored in addition to chimeric antigen receptor (CAR) T-cell therapy which is also being investigated in clinical trials as a potential treatment option.

Progressive transformation of germinal centers or nodular lymphocyte-predominant Hodgkin lymphoma? Issues of differential diagnosis: a clinical case

Progressive transformation of germinal centers (PTGC) is a benign reactive lymphadenopathy, which can be an independent disease or occur in association with other lymphomas, the most common variant of which is nodular lymphocyte predominant Hodgkin lymphoma (NLPHL). To date, it has not been definitively clarified how PTGC and NLPHL are interconnected, despite the abundance of works presented on this topic. PTGC may precede NLPHL, occur synchronously with it, or develop after a course of therapy in patients with NLPHL. Despite similar clinical and morphological features, the approach to the treatment and management of patients is different. In the case of NLPHL, one of the therapeutic options is chemotherapy, which is not used in patients with PTGC. This article presents a clinical case of partial lymph node lesion of NLPHL associated with PTGC, on the example of which the main issues of differential diagnosis of PTGC and NLHLP will be considered. The patient's parents gave their consent to the use of their child's data, including photographs, for research purposes and in publications.

Machine Learning Logistic Regression Model for Early Decision Making in Referral of Children with Cervical Lymphadenopathy Suspected of Lymphoma

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. The outcome groups were classical Hodgkin lymphoma (cHL), nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL), non-Hodgkin lymphoma (NHL), other malignancies, and a benign group. We integrated the best univariate predicting factors into a multivariate, machine learning model. Logistic regression allocated each variable a weighing factor. The model was tested in a different patient cohort (n = 60). We report a 12-factor diagnostic model with a sensitivity of 95% (95% CI 89-98%) and a specificity of 88% (95% CI 77-94%) for detecting cHL and NHL. Our 12-factor diagnostic scoring model is highly sensitive and specific in detecting high-grade lymphomas in children with cervical lymphadenopathy. It may enable fast referral to a pediatric oncologist in patients with high-grade lymphoma and may reduce the number of referrals and unnecessary invasive procedures in children with benign lymphadenopathy.

IMP3 Immunohistochemical Staining: A Valuable Tool in the Differential Diagnosis of Nodular Lymphocyte Predominant Hodgkin Lymphoma and T-Cell/Histiocyte-Rich Large B-Cell Lymphoma

IMP3 Immunohistochemical Staining: A Valuable Tool in the Differential Diagnosis of Nodular Lymphocyte Predominant Hodgkin Lymphoma and T-Cell/Histiocyte-Rich Large B-Cell Lymphoma

IMP3 Immunohistochemical Staining: A Valuable Tool in the Differential Diagnosis of Nodular Lymphocyte Predominant Hodgkin Lymphoma and T-Cell/Histiocyte-Rich Large B-Cell Lymphoma

IMP3 Immunohistochemical Staining: A Valuable Tool in the Differential Diagnosis of Nodular Lymphocyte Predominant Hodgkin Lymphoma and T-Cell/Histiocyte-Rich Large B-Cell Lymphoma