Mature aggressive B-cell lymphomas, such as Burkitt lymphoma (BL) and Diffuse large B-cell lymphoma (DLBCL), show variations in microRNA (miRNA) expression. The entity of High-grade B-cell lymphoma with 11q aberration (HGBCL-11q) shares several biological features with both BL and DLBCL but data on its miRNA expression profile are yet scarce. Hence, this study aims to analyze the potential differences in miRNA expression of HGBCL-11q compared to BL and DLBCL. We evaluated the expression profiles of 2083 miRNAs in 25 HGCBL-11q, 7 BL, 131 DLBCL, and tonsils using the HTG EdgeSeq miRNA whole transcriptome assay. Uniform manifold approximation and projection (UMAP) and differential gene expression analyses based on DESeq2 were carried out. UMAP analysis of miRNA expression did not reveal distinct groups among the studied lymphomas. However, differential gene expression investigations detected sets of overexpressed miRNAs in HGBCL-11q when compared to BL (miR-9-3p, miR-9-5p, miR-3919, miR-129-1-3p, miR-129-2-3p, miR-331-3p, miR-196b-5p, and miR-28-5p) and DLBCL (miR-3919, miR-1290, miR-4538, and miR-4791), respectively. Notably, miR-3919 showed heterogeneous but significantly higher expression (p-value < 0.001) in HGBCL-11q than in both, BL and DLBCL. We identified a group of differentially expressed miRNAs between HGBCL-11q vs. BL and DLBCL, with miR-3919 as the most commonly and recurrently overexpressed miRNA in HGBCL-11q.

Follicular lymphoma is one of the most common non-Hodgkin’s lymphomas in adults. One of the rather rare variants of follicular lymphoma is pediatric follicular lymphoma. This variant, despite the name, is diagnosed not only in children, but also among young adults. Pediatric follicular lymphoma is characterized by early (I, II) stages, the absence or weak BCL2 expression, and the predominant absence of t(14;18)(q32;q21) translocation. Treatment tactics vary widely from radical surgical tumor resection with subsequent follow-up to chemoimmunotherapy with rituximab.This article presents a clinical case of advanced pediatric follicular lymphoma (stage III) in a 5-year-old patient. Using a combined treatment approach (surgery followed by immunochemotherapy) allowed to achieve a complete metabolic response which lasts more than a year.

Abstract High-grade B-cell lymphoma with 11q aberrations (HGBL-11q) is a type of aggressive B-cell lymphoma that shares morphological, immunophenotypic and gene expression features with Burkitt lymphoma (BL). HGBL-11q and BL differ in terms of the MYC translocation and typical landscapes of structural and small nucleotide variants. HGBL-11q harbours the name-giving 11q aberrations, and its mutational landscape is more similar to diffuse large B-cell lymphoma (DLBCL) than to BL. Despite these differences, BL and HGBL-11q have similar gene and protein expression profiles to normal B-cells in the germinal center (GC) dark zone (DZ). Recent single-cell RNA sequencing (scRNA-seq) studies have identified various subpopulations within the GC; in this study we aimed to explore the differences between HGBL-11q and BL based on the expression patterns of these different subpopulations within the GC. Transcriptional profiling by HTG EdgeSeq Pan B-Cell Lymphoma Panel, which includes 298 genes, was applied to FFPE sections of 7 BL, 26 HGBL-11q, 132 DLBCL, and two tonsils as controls. The panel was validated against RNAseq data available from overlapping samples of the ICGC MMML-Seq cohort, leaving 206 genes for further analyses.[OG(1] [EC2] [r3] From these, 60 genes overlapped with those included in expression signatures of GC subpopulations described in published scRNA-seq datasets. We investigated the ability of this reduced gene set to describe GC subpopulations in silico and investigated those gene sets proving robustness in the lymphomas dataset. Based on the expression of the 60 genes associated with specific GC populations, we also performed unsupervised clustering analyses using principal component analysis, t-distributed Stochastic Neighbor Embedding, and Uniform Manifold Approximation and Projection, as well as supervised DESeq2 analyses to identify differentially expressed genes. Out of all samples, 25 HGBL-11q and 50 DLBCL revealed a GC B-cell signature, along with all BLs. A comparison of the GC subpopulation expression patterns revealed heterogeneity in GCB-type DLBCL with a prevalent light zone expression pattern, while BL and HGBL-11q exhibited a quite homogenous pattern. BL showed a predominant DZ pattern, whereas HGBL-11q had high expression of DZ-associated genes similar to BL but also showed a considerable expression of genes associated with intermediate GC-associated genes. Cluster analysis with the 60 genes derived from the overlap with the GC population signatures showed that HGBL-11q segregates separately from BL and DLBCL notwithstanding the shared DZ pattern. Differential expression analysis confirmed that genes associated with intermediate GC B-cells, regardless of their chromosomal location, were higher in HGBL-11q compared to BLs, whereas ID3 was the top upregulated gene in BL as compared to HGBL-11q. In conclusion, HGBL-11q exhibits a DZ-related gene expression pattern similar to, but nevertheless distinct from, BL particularly in genes associated with GC intermediate B-cells. Citation Format: Emil Chteinberg, Gioia Di Stefano, Henry Loeffler-Wirth, Annette Staiger, Sina Hillebrecht, Rabea Wagener, Susanne Bens, Kathrin Oehl-Huber, Sonja Dahlum, Dmitriy Abramov, Birgit Burkhardt, Abner Louissaint Jr, Katrin Kurz, Heike Horn, Anja Mottok, Raffaella Santi, Ilske Oschlies, Wolfram Klapper, Kristian Schafernak, Yanming Zhang, Andreas Rosenwald, Hans Binder, Megan S. Lim, German Ott, Lorenzo Leoncini, Coral Del-Val, Reiner Siebert. High-grade B-cell lymphomas with 11q aberrations show a dark zone expression profile similar to Burkitt lymphoma but with additional extension towards intermediate germinal center B-cells [abstract]. In: Proceedings of the Blood Cancer Discovery Symposium; 2024 Mar 4-6; Boston, MA. Philadelphia (PA): AACR; Blood Cancer Discov 2024;5(2_Suppl):Abstract nr P35.

ALK-positive anaplastic large cell lymphoma is a mature T-cell lymphoma characterized by translocations that involve the ALK receptor tyrosine kinase coding gene. This illness is known to almost exclusively affect children and young adults. The biology of ALK-positive anaplastic large cell lymphoma is fairly well researched today, with recent studies focusing on the histogenesis of this neoplasm. In this review, we analyze the existing world literature data on the etiology and pathogenesis of this disease.

X-linked agammaglobulinemia (XLA) is a primary immunodeficiency (PID) characterized by defective B cell maturation, low serum immunoglobulin concentrations and recurrent infectious episodes. The disease is caused by defects of the BTK gene, which encodes Bruton's tyrosine kinase. Along with frequent infections, autoimmune complications of XLA are an important issue. Inflammatory bowel disease-like syndromes (IBD) represent a special group of complications of XLA, their pathophysiology largely unknown. Materials and methods used: Authors conducted a retrospective data analysis of 56 patients with XLA followed at the National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev (Moscow, Russia) in 2011-2022. Results: IBD-like disease was diagnosed in 11 of 56 (19.6%) patients with XLA, based on medical history, endoscopic picture, histological and laboratory studies. The median onset of the first clinical manifestations of IBD was 7 years (2;14). The most common symptoms were weight loss (7/11, 64%), chronic diarrhea (7/11, 64%) and recurrent abdominal pain (5/11, 45%). Blood in the stool was detected in a quarter of the patients (3/11, 27%). Upon implementation of anti-inflammatory therapy, the majority of patients available for assessment (7/10, 70%) demonstrated improvement. Complete response to therapy with mesalazine was achieved in 2 patients out of 9 who used it (22%). Complete response to treatment with adalimumab was observed in 1 out of 4, with infliximab in 1/3, and with vedolizumab in 1/2. A single patient had refractory IBD without response to all treatment modalities, which served as an indication for allogeneic hematopoietic stem cell transplantation (HSCT), which unfortunately resulted in fatal outcome due to early post-transplant complications. Conclusion: intestinal symptoms in XLA require special vigilance and a multidisciplinary approach. In the study group, the use of variable anti-inflammatory therapy in the majority of patients was shown to be effective and did not lead to the development of additional infectious complications. Analysis of a larger number of patients with XLA and IBD would allow standardization of anti-inflammatory therapy selection in this complex group.

In recent years, there has been a trend towards de-escalation of therapy in patients with early stages of nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) which enables reduction in the frequency of late effects of chemo- and radiation therapy while still maintaining their effectiveness. Patients with stage I NLPHL only require excisional biopsy of lymph nodes. If complete remission cannot be achieved by surgery alone or if patients have stage II NLPHL, 3 cycles of low-dose CVP (cyclophosphamide, vinblastine, prednisolone) chemotherapy are administered. In some cases, patients show incomplete response to therapy with subsequent progression of the disease. Hence, the search for factors of unfavorable clinical course of NLPHL still continues, with an immunoarchitectural pattern potentially being one of them. Here, we aimed to compare clinical features, treatment responses and relapse rates in patients with NLPHL based on the type of an immunoarchitectural pattern. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. In our study, we included a cohort of 49 patients (39 boys, 10 girls) aged 2 to 18 years (median age: 10 years) with diagnosed NLPHL who were divided into 2 groups based on histological features of the disease: typical patterns (n = 21, 42.9 %) and atypical patterns (n = 28, 57.1 %). The two groups were compared using the exact Fisher test. Thirty-three patients had early stage I–II disease at baseline, 14 patients had stage III disease, and 2 patients were diagnosed with stage IV lymphoma affecting the liver and lungs in one case and bones in the other. Clinical characteristics (such as disease stage, B symptoms, the involvement of mediastinal and intra-abdominal lymph nodes) didn’t vary much between the groups, the only exception being the presence/absence of bulky disease (≥ 6 cm) (p = 0.0064). A higher rate of partial response to therapy and disease progression frequency were revealed in the group of atypical patterns (typical: n = 1/21, 4.8 % vs atypical: n = 14/28, 50 %; p = 0.00061). This group was also characterized by a higher relapse rate (typical patterns: n = 1/21, 4.8 % vs atypical: n = 5/28, 17.9 %; p = 0.219). The overall survival rate was 100%, with a median follow-up of 28 (3–108) months. In our study, we revealed a higher incidence of adverse outcomes in the patients with atypical NLPHL patterns compared to the group with typical patterns. The prognostic value of immunoarchitectural patterns needs to be explored more thoroughly, as they have the potential to become one of the criteria for risk stratification of patients with NLPHL.

Today, results of allogeneic hematopoietic stem cell transplantation (allo-HSCT) are very encouraging but nevertheless, 25–75% of patients still develop complications, and mortality rates reach as high as 10–19%. Two major complications of allo-HSCT are graft-versus-host disease (GVHD) and viral infections. The diagnosis of intestinal GVHD remains a challenge. Incorrect interpretation of gastrointestinal lesions may lead to grave consequences. The development of endoscopic criteria for GVHD is a major focal point in foreign literature. In our study, we included 33 patients aged 1–17 years with signs of enterocolitis and suspected isolated intestinal GVHD who had received allo-HSCT at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of Russia between 2020 and 2023. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of Russia. All the patients underwent white-light colonoscopy with i-scan and, if necessary, chromoendoscopy; as well as colonic mucosal biopsy for further histopathological evaluation and intestinal infection testing. Statistical analysis was performed using the IBM SPSS Statistics 23 software. Viruses were identified in the colonic mucosal samples of 84.8% of the patients. The most common pathogens were: HHV-6 (in 50% of the cases), norovirus (46.4%) and adenovirus (35.7%). The high incidence of colonic viral infections in the children with GVHD is likely the result of virus reactivation during intensive immunosuppressive therapy following allo-HSCT. In 78.8% of the study patients, the colonic mucosa had orange-peel appearance, which led us to consider such mucosal changes to be the main criterion for the endoscopic diagnosis of GVHD in children after allo-HSCT, regardless of the presence or absence of viral infections. Our study demonstrated a fairly high effectiveness of the chromoendoscopy advanced imaging technology. Further research on ways to improve methods for GVHD differential diagnosis is required.

BCR::ABL/Ph-negative chronic myeloproliferative neoplasms (CMPN) in children differ from those in adults in clinical manifestations and genetic alterations. Taking into account the well-known physiology of hematopoiesis in children, it seems important to compare the histological features of CMPN in pediatric patients with the criteria for the diagnosis of these diseases in adults specified in the World Health Organization (WHO) classification. In pediatric practice, the interpretation of changes in hematopoiesis in patients with CMPN without any established driver mutation has a particular importance for differential diagnosis with secondary thrombocytosis and erythrocytosis. For our analysis, we used bone marrow trephine biopsy specimens from the biobank of the Pathology Department of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation. They had been obtained between 2016 and 2023 from 70 patients for initial histological examination. The final clinical diagnosis for these patients was CMPN. The frequency of the most common histological changes in hematopoiesis was assessed retrospectively. We compared our results with the data from the WHO classification, analysed the differences in morphological changes in the subgroups of patients with essential thrombocythemia with an established mutation or without it, assessed the relationship between the morphological changes and clinical symptoms of CMPN. The changes in hematopoiesis in children with CMPN are predominantly similar to those in adults, however there are differences in the morphology of megakaryocytes (scarcity of giant cells with hypersegmented nuclei (staghorn-like), an increased number of small and naked nuclei cells). In addition, bone marrow cellularity assessment has a low diagnostic value in differentiating between essential thrombocythemia and polycythemia vera in children. There are no differences in morphology in the subgroups of patients with essential thrombocythemia with an established mutation or without it. No statistically significant association between clinical symptoms of the disease and any of the morphological features of CMPN was found. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation.

ALK-positive anaplastic large cell lymphoma is a rare T-cell lymphoma with ALK gene rearrangement that develops in children and young adults. The disease almost always affects the lymph nodes, and extranodal areas are also frequently involved. This article describes two cases of atypical localization of ALK-positive anaplastic large cell lymphoma with involvement of the paranasal sinuses.

Rosai–Dorfman disease is the most frequent variant of non-Langerhans cell histiocytosis. Local forms can be resected or irradiated. If the process involves multiple organs, systemic chemotherapy can cure some patients. This article includes literature review and a case report of a 34-year-old patient with multifocal, multisystemic form of Rosai–Dorfman disease with bone and pleural involvement. The diagnosis was based on histological, immunohistochemical, and molecular studies of tumor tissue. Since November 2021, 6 courses of chemotherapy with cladribine and 8 infusions of zolendronic acid were carried out with achievement of durable remission. The tolerance was acceptable.

Burkitt lymphoma (BL) is one of the most common types of non-Hodgkin lymphoma in children. The application of modern risk-adapted treatment regimens has resulted in 85–90 % survival rates in affected patients; however, prognosis still remains poor in case of relapsed/refractory disease. In standard protocols, patients were stratified into risk groups based primarily on disease stage and extent and lactate dehydrogenase levels. Mutations in the TP53 gene are associated with a poor prognosis in many tumors, and lately there have been reports that TP53 status may have prognostic value in pediatric BL. We analyzed therapy outcomes in patients treated in accordance with the B-NHL-2010M protocol according to their TP53 mutational status. We discovered that the 5-year event-free and overall survival rates in the patients with TP53 mutations were 45.3 % and 47.1 % respectively, versus 97.9% and 97.9% in those without TP53 mutations (p < 0.001). Hence, TP53 mutational status is an important prognostic marker in pediatric patients with BL and should be utilized in future protocols. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology.

T-lymphoblastic lymphoma (T-LBL) is one of the most common non-Hodgkin lymphomas in children. According to the 2022 WHO classification, T-LBL and acute T-lymphoblastic leukemia are considered as a single disease since they both have T-cell precursors as a morphological substrate. In recent years, some progress has been made in the treatment of this disease, but the prognosis for relapses and refractory cases remains extremely unfavorable. One of the promising areas that can increase the effectiveness of therapy is the use of new treatment approaches that consider the molecular and biological features of this tumor. This review examines in detail the molecular aspects of the pathogenesis of T-LBL.

The aim of our study is to evaluate the prognostic value of the morphological types of ALK-positive anaplastic large cell lymphoma (ALK+ ALCL) in children. We performed a retrospective analysis of data on 81 cases of pediatric ALK+ ALCL which had been diagnosed in 2011–2022. All patients and/or their legal representatives signed voluntary informed consent for participation in the study, as well as for biological material testing. The analysis of medical records was carried out in accordance with the internal rules of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthсare of the Russian Federation, developed and approved by the Independent Ethics Committee of the Center. The patients received treatment according to the standard protocol. We assessed overall (OS) and event-free (EFS) survival depending on the morphological type of the tumor. The median follow-up was 55.6 months. Three-year and 5-year OS rates were 81.9% and 79.8%, respectively. Three-year and 5-year EFS rates were 59.6% and 56.0%, respectively. There were no statistically significant differences in OS and EFS between the common and non-common morphological types of ALK+ ALCL. Better survival rates were observed in the patients with a lymphohistiocytic variant. We found statistically significant differences in OS (p = 0.031) and EFS (p = 0.002) between the cases with a small cell component and without it. The results suggest that ALK+ ALCL with small cell morphology has a more aggressive course in children. Validation in larger patient cohorts and further study of the biology of different morphological types are needed to develop stratified treatment approaches.

Introduction. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a cell therapy method that is widely used in hematological malignancies, solid tumors, hemoglobinopathies, primary immunodeficiencies, and other congenital diseases. One of the serious complications after allo-HSCT is graft-versus-host disease (GVHD). Acute GVHD affects the skin, liver, and gastrointestinal (GI) tract. In this connection, endoscopic diagnosis acquires special importance in the comprehensive diagnosis of gastrointestinal GVHD. The aim of the work was to determine the relationship between the severity of the clinical course of acute GVHD, visualization of villous atrophy during ileocolonoscopy and morphological changes in the mucous membrane of the terminal ileum in children after allo-HSCT. Materials and methods. There was conducted a retrospective study including forty eight 9 months–8 years patients who underwent allo-HSCT between 2020 and 2023. Depending on the presence and severity of atrophy. All 48 children were divided into four groups: group 1 included 18 (37.5%) patients with signs of pronounced atrophy according to endoscopy; group 2 included 6 (12.5%) children with mild villous atrophy; Group 3 included 7 (14.6%) patients without atrophy, but with signs of ileitis; group 4 was formed by 17 (35.4%) children without signs of inflammation of the mucous membrane of the terminal ileum. All subjects underwent ileocolonoscopy using Pentax high-resolution endoscopes (Japan), with a biopsy of the mucous membrane of the colon and ileum. Statistical data processing was performed using IBM SPSS Statistics 23 software. Results. When comparing endoscopy data with the clinical picture, there was noted a tendency to identify a more severe degree of acute GVHD in children from groups 1 and 2 with endoscopic signs of villous atrophy. Acute grade 3 GVHD was diagnosed only in children with endoscopic signs of villous atrophy — in 16.7%. In the 3rd and 4th groups of patients, there was a tendency towards more frequent detection of acute GVHD grade 0, compared to children with signs of atrophy (25% versus 8.3%); however, the differences were statistically insignificant (p > 0.05). According to histology, in the majority of patients in groups 1 and 2 (21 out of 24/87.5%), ileoscopy revealed “false” villous atrophy. In the 3rd group, not a single child showed signs of atrophy confirmed histologically, which was consistent with expectations. In 13 out of 17 (76.5%) patients of the 4th group, histological examination showed no pronounced signs of inflammation. In 3 (17.6%) children of the 4th group there were no morphological changes in the ileal mucosa. Conclusion. The clinical significance of atrophy and the prognostic value of this criterion remain controversial, which indicates to the need for continued research in this area.

Primary cutaneous lymphomas are quite rare in children. Clinical and histopathological manifestations of these diseases in children differ significantly from those in adults. Due to their rarity and complex clinical presentation, diagnosis may take long time. Mycosis fungoides (MF) is the most commonly diagnosed form of primary cutaneous lymphomas in childhood. There are no clinical guidelines for the treatment of children. Literature data on MF variants in children are scarce; the largest study includes 34 patients who were diagnosed on average 4 years after the onset of the first symptoms. In the present article we describe a clinical case of MF in an 11-year-old child with an 8-year history of multiple lesions of the skin and scalp. The patient's parents gave their consent to the use of their child's data, including photographs, for research purposes and in publications. The aim of our article is to demonstrate the problems in the diagnosis of the disease, especially at an early stage, because its symptoms may be similar to those of many common pediatric inflammatory skin conditions.

Nijmegen breakage syndrome (NBS) and ataxia-telangiectasia (AT; Louis–Bar syndrome) are primary immunodeficiencies (PID) associated with chromosome instability and DNA repair defects that predispose individuals to an increased risk of various malignancies. In our study, we retrospectively analyzed clinical characteristics and outcomes of 28 cancer cases in 14 patients with AT and 10 patients with NBS who had been treated at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology between January 2007 and December 2022. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. The most common type of malignancy was mature B-cell non-Hodgkin lymphoma (B-NHL) (42%), with diffuse large B-cell lymphoma (DLBCL) accounting for 91% of all B-NHL cases. Other cases included T-cell acute lymphoblastic leukemia (ALL) ( n = 3), B-cell ALL ( n = 2), Hodgkin lymphoma ( n = 3), NK/T-cell lymphoma ( n = 1), T-cell lymphoblastic lymphoma ( n = 1), peripheral T- cell lymphoma ( n = 2), medulloblastoma ( n = 1) epithelioid rhabdomyosarcoma ( n = 1), T-cell prolymphocytic leukemia ( n = 2). A total of 4 patients were diagnosed with second malignancies (2 children with AT and 2 children with NBS. The diagnosis of PID was suspected or confirmed before the initiation of cancer therapy in 62% of AT patients and in 100% of NBS patients. Treatment was given in accordance with standard protocols with chemotherapy dose modifications. A total of 93% of patients with AT and 80% of patients with NBS required dose reduction. The level of response was quite high: 81% of patients with AT and 58% of patients with NBS achieved complete remission. According to our data, the use of reduced-dose chemotherapy regimens helps to achieve an acceptable toxicity profile without reducing the overall effectiveness of treatment.

The effectiveness of treatment for children with B-cell non-Hodgkin lymphomas (B-NHL) has reached 85–90% after the introduction of modern risk-adapted treatment regimens that involve risk group stratification based on tumor stage. Bone marrow involvement is traditionally evaluated using quantitative morphological analysis of tumor cells which has, however, a lower sensitivity compared to molecular genetic methods. In our study, we used next generation sequencing (NGS) to identify tumor-specific V-(D)/J-rearrangements of immunoglobulin genes which can be used as a marker for the evaluation of minimal disseminated disease (MDD) in children with B-NHL. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. Here we demonstrated that NGS allows detection of bone marrow involvement at a sensitivity of 10–6 in patients with Burkitt lymphoma, in whom standard morphological analysis failed to reveal the presence of tumor cells. The detection of molecular MDD can improve tumor staging and risk stratification in children with B-cell non-Hodgkin lymphomas.

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.