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Targeted Therapies for the Treatment of Pediatric Non-Hodgkin Lymphomas: Present and Future.

Pediatric Non-Hodgkin Lymphomas (NHL) are a diverse group of malignancies and as such treatment can vary based on the different biological characteristics of each malignancy. Significant advancements are being made in the treatment and outcomes of this group of malignancies. This is in large part due to novel targeted drug therapies that are being used in combination with traditional chemotherapy. Here, we discuss several new lines of therapy that are being developed or are in current use for pediatric patients with NHL.

Selected Abstracts from the 12th Annual Meeting of the Clinical Immunology Society: 2021 Virtual Annual Meeting: Immune Deficiency and Dysregulation North American Conference.

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From Biology to Treatment of Monoclonal Gammopathies of Neurological Significance.

Monoclonal gammopathy and peripheral neuropathy are common diseases of elderly patients, and almost 10% of patients with neuropathy of unknown cause have paraprotein. However, growing evidence suggests that several hematological malignancies synthesize and release monoclonal proteins that damage the peripheral nervous system through different mechanisms. The spectrum of the disease varies from mild to rapidly progressive symptoms, sometimes affecting not only sensory nerve fibers, but also motor and autonomic fibers. Therefore, a multidisciplinary approach, mainly between hematologists and neu

Papillary thyroid carcinoma in a patient with long-standing chronic lymphocytic leukemia: A case report.

The synchronous occurrence of papillary thyroid carcinoma (PTC) and chronic lymphocytic leukemia (CLL) is rare and poses diagnostic challenges, particularly in distinguishing metastatic thyroid disease from hematological lymphadenopathy. The present report documents the case of a 64-year-old female patient with long-standing CLL who presented with a malignant thyroid nodule accompanied by multiple enlarged cervical lymph nodes. A diagnosis was established through integrated immunohistochemistry, flow cytometry and multimodal imaging. After the diagnosis of synchronous PTC and CLL/SLL was confi

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… These findings support a clonal mature B-cell lymphoproliferative disorder compatible with CLL/SLL. Figure 2. Peripheral blood flow cytometry analysis. In total, ~80.06% abnormal mature B lymphocytes were identified, showing κ light chain restriction. … Therefore, in cases of thyroid carcinoma with dense monomorphic lymphocytic infiltration, clinicians should maintain a high index of suspicion for concomitant lymphoproliferative disorders, such as CLL. …

Автономная трансплантация стволовых клеток с помощью гематопоиетической гематомии для множественных склерозов в Соединенном Королевстве: A 20-year Ретроспективный анализ деятельности и гематологических результатов Британского общества трансплантации крови и мозга и клеточной терапии (BSBMTCT).

Автологическая трансплантация стволовых клеток (AHSCT) была разработана в качестве средства лечения множественных склерозов (MS) с тех пор. 1995. Соединенное Королевство one :: из наиболее активных стран Европы, осуществляющих АХСХТ для НР. Мы сообщаем об опыте Соединенного Королевства в области AHSCT для MS 364 :: пациенты, имеющие MS, которые лечатся AHSCT между 2002 и 2023. Мы представляем данные о смертности, связанной с трансплантацией (ТМР), осложнениях и эффективности АХСПП, определяемых расширенной шкалой показателей инвалидности (ЭДСС) в условиях бесперебойного выживания (ПФС) 2 годы

Summary for clinicians: ERS guidelines on pulmonary alveolar proteinosis.

Pulmonary alveolar proteinosis (PAP) is a rare lung disease caused by accumulation of surfactant in the alveoli, leading to debilitating respiratory symptoms and impaired gas exchange. The recent European Respiratory Society guidelines provide evidence-based recommendations for its diagnosis and management. Autoimmune PAP (aPAP) is the most common form, driven by granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies. Recommended diagnostic tools include bronchoalveolar lavage and quantitative GM-CSF antibody testing. Whole lung lavage and inhaled GM-CSF are first-line treatm

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… Some of these are caused by mutations in the genes that are involved in surfactant metabolism or genes that encode surfactant, others are caused by occupational exposures and secondary diseases such as lymphoproliferative disorders [ 4 ]. …

An update on the diagnosis and management of the polyneuropathy of POEMS syndrome.

POEMS syndrome is a rare, chronic, disabling paraneoplastic disorder characterized by peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma cells disorder and skin changes. Diagnosis relies on the fulfillment of a set of clinical criteria of which polyneuropathy and a monoclonal plasma cell dyscrasia are early and essential features. Treatment may be either local or systemic and is aimed at the monoclonal plasma cell disorder. Our knowledge of the pathogenesis underlying the POEMS syndrome has advanced greatly over the past years, favoring an important progression in the recog

Controversies about immunoglobulin replacement therapy in HSCT recipients with hypogammaglobulinemia.

The efficacy of immunoglobulin replacement therapy (IgRT) has been demonstrated for primary immune deficiency diseases and hematological malignancies such as chronic lymphocytic leukemia (CLL) or multiple myeloma with hypogammaglobulinemia. Clinical development of anti-B cell therapies including a monoclonal antibody, bispecific antibody, or chimeric antigen receptor T-cell therapy which could result in severe hypogammaglobulinemia accelerates the argument of prophylactic use of IgRT. Clinical guidelines for CLL describe immunoglobulin administration in patients with a low IgG who have experie

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… According to the Cochrane Database of Systematic Reviews for HSCT and non-HSCT cases, no survival benefit associated with IgRT was confirmed, whereas a subgroup-analysis with lymphoproliferative disorders cohort showed a lower incidence of clinically and microbiologically-documented infection by IVIG … Compagno N Cinetto F Semenzato G Agostini C Subcutaneous immunoglobulin in lymphoproliferative disorders and rituximab-related secondary hypogammaglobulinemia: a single-center experience in 61 patients Haematologica 2014 99 1101 6 10.3324/haematol.2013.101261 24682509 PMC4040915 20. …

Waldenström macroglobulinemia: biology, genetics, and therapy.

Waldenström macroglobulinemia (WM) is a distinct clinicopathologic entity characterized by the presence of a lymphoplasmacytic lymphoma, a non-Hodgkin lymphoma, and IgM monoclonal gammopathy. WM is an indolent, uncommon malignancy mostly affecting the elderly. Patient outcomes have modestly improved since the introduction of rituximab to conventional cytotoxic chemotherapy more than 20 years ago. However, the pivotal discovery of the somatic MYD88 L265P mutation, harbored by most patients with WM, and the somatic CXCR4 WHIM mutations, similar to germline CXCR4 mutations seen in the warts, hypo

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… Features required for the diagnosis of WM include 1) presence of an IgM monoclonal gammopathy of any size in serum, 2) bone marrow biopsy findings showing LPL infiltration, and 3) exclusion of other lymphoproliferative disorders, including CLL and MCL. … disorders using conventional and quantitative allele-specific polymerase chain reaction Blood 2013 121 11 2051 2058 23321251 10.1182/blood-2012-09-454355 PMC3596964 32 Ngo VN Young RM Schmitz R Oncogenically active MYD88 mutations in human lymphoma Nature 2011 470 7332 115 119 21179087 10.1038/nature09671 …

Двустороннее участие грудных вскармливаний в множестве миелом: доклад о реалистическом деле.

Грудная плазмоцитома представляет собой редкое дополнительное проявление множественной миеломы (ММ). Этот случай представляет собой первичную экстрамульгальную плазмоцитому и переход к MM с участием груди после 12 годы обнаружения болезни. Пациент - это 54-year-old Женщина, у которой изначально был правый патологический перелом плечевой кости, оказавшийся основной дополнительной плазмоцитомой. Она получила химиотерапию и получила ремиссию. Six Спустя несколько лет болезнь повторилась как масса в грудине, которая была обработана радиацией и химиотерапией, но без улучшения. Затем болезнь перешла

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… As US and mammography findings are not adequate for diagnosis, the differential diagnosis for benign masses, other lymphoproliferative disorders, and primary carcinoma largely depends on histopathological evaluation [ 5 ]. …

MicroRNA-17-92a-1 Host Gene (MIR17HG) Expression Signature and rs4284505 Variant Association with Alopecia Areata: A Case-Control Study.

Accumulating evidence indicates the implication of microRNAs (miRs) in cutaneous and hair follicle immunobiology. We evaluated, for the first time, the miR-17-92a-1 cluster host gene (MIR17HG) expression in peripheral blood of 248 unrelated alopecia areata (AA) patients compared to 244 matched controls using Real-Time qPCR. We also tested its association with different rs4284505A>G genotypes (based on TaqMan allelic discrimination PCR) and the available clinical data. The adjusted odds ratio (OR) and 95% confidence interval (CI) were calculated for each genetic association model. The upregulat

Daratumumab-based treatment of monoclonal gammopathy-associated angioedema due to acquired C1-inhibitor deficiency.

Daratumumab-based treatment could control severe, treatment-refractory, life-threatening angioedema due to acquired C1-inhibitor deficiency associated with monoclonal gammopathy.

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… Found in 1% to 2% percent of adults, it is typically asymptomatic and often diagnosed through the incidental finding of a serum monoclonal protein (M-protein) after diagnostic exclusion of lymphoproliferative disorders. …

MASTering systemic mastocytosis: Lessons learned from a large patient cohort.

BACKGROUND: Systemic mastocytosis (SM), a rare condition affecting about 32,000 individuals in the United States, is often misdiagnosed or underdiagnosed owing to its nonspecific symptoms and the need for invasive biopsies. OBJECTIVE: Our aim was to identify, classify, and characterize the natural history of patients with SM. METHODS: In a retrospective cohort study, administrative data from a large managed care organization was used to identify patients with confirmed SM, based on World Health Organization criteria. Demographic data, delay to diagnosis, disease progression, and health care

Paediatric intestinal pseudo-obstruction: a scoping review.

Paediatric intestinal pseudo-obstruction (PIPO) encompasses a group of rare disorders in which patients present with the clinical features of bowel obstruction in the absence of mechanical occlusion. The management of PIPO presents a challenge as evidence remains limited on available medical and surgical therapy. Parenteral nutrition is often the mainstay of therapy. Long-term therapy may culminate in life-threatening complications including intestinal failure-related liver disease, central line thrombosis and sepsis. Intestinal transplantation remains the only definitive cure in PIPO but is a

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… Of the latter, monitoring for cytomegalovirus and Epstein-Barr virus infections is imperative to minimise predisposition to associated lymphoproliferative disorders [ 58 ]. Furthermore, the road to transplantation is not without issues. …

Genomic instability and genetic heterogeneity in aging: insights from clonal hematopoiesis (CHIP), monoclonal gammopathy (MGUS), and monoclonal B-cell lymphocytosis (MBL).

Aging is a multifaceted process characterized by a gradual decline in physiological function and increased susceptibility to a range of chronic diseases. Among the molecular and cellular mechanisms driving aging, genomic instability is a fundamental hallmark, contributing to increased mutation load and genetic heterogeneity within cellular populations. This review explores the role of genomic instability and genetic heterogeneity in aging in the hematopoietic system, with a particular focus on clonal hematopoiesis of indeterminate potential (CHIP), monoclonal gammopathy of undetermined signifi

Муриновая корреляция саркомы Эпштейна-Барра и саркомы Капози (MHV-68) представляет собой ценную модель для оценки чувствительности к антивирусным препаратам и взаимодействия в комбинаторных наркотиках.

Человеческие герпесвирусы представляют собой основные человеческие патогены. Особый интерес представляют члены подсемейства < < Герпесвирина > > , поскольку они вызывают хронические заболевания, такие, как рак, изменяя клеточные процессы и вводя клетки отдыха в непрерывно расширяющуюся фазу. Кроме того, в центре внимания постоянно находятся аутоиммунные заболевания, такие, как множественный склероз. Прототипический вирус человека - герпесвирус - вирус Эпштейна - Барра (ЭБВ) оказался трудно проанализировать в целях развития противовирусной терапии из-за проблем, связанных с распрост

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… Beyond acute illness, EBV establishes lifelong persistence and is associated with a spectrum of malignancies, including Burkitt’s and Hodgkin’s lymphomas, nasopharyngeal carcinoma, gastric cancer, and post-transplant lymphoproliferative disorders, particularly in immunocompromised patients ( Chinna et …

Whole brain radiotherapy combined with intrathecal liposomal cytarabine for leptomeningeal metastasis-a safety analysis and validation of the EANO-ESMO classification.

BackgroundAlthough there is no proven standard therapy for leptomeningeal metastases (LM), treatment often includes intrathecal chemotherapy combined with whole brain radiation therapy (WBRT). Little is known about the toxicity of such combination therapies. We performed a retrospective safety analysis for the combination of intrathecal liposomal cytarabine with WBRT in patients with LM and validated the EANO-ESMO (European Association of Neuro-oncology-European Society for Medical Oncology) classification in this unique cohort.MethodsTreatment toxicities in patients diagnosed with LM between

Paraneoplastic Syndrome After Kidney Transplantation: Frequency, Risk Factors, Differences to Paraneoplastic Occurrence of Glomerulonephritis in the Native Kidney, and Implications on Long-Term Kidney Graft Function.

Posttransplant malignancies are an important complication of solid organ transplantation. Kidney transplant recipients are at particularly high risk of cancer development. The most relevant risk factors of carcinogenesis are the use of immunosuppressive agents and oncogenic viral infections. Additionally, immune dysregulation caused by these factors may predispose to various types of organ damage. Paraneoplastic glomerular diseases are one of the most interesting and understudied cancer manifestations. The appropriate diagnosis of paraneoplastic glomerular damage can be challenging in kidney t

Global Panoramic analysis of clinical research in cell therapy: clinical trial landscape, marketed products, and regulatory trends.

IntroductionCell therapy is a vital field in modern medicine. This review assesses its global clinical development landscape, approved products, and regional regulatory characteristics.MethodsA comprehensive search and analysis of global clinical trial databases (e.g., https://clinicaltrials.gov/ClinicalTrials.gov), regulatory agency announcements, and relevant literature up to October 2025 was conducted. Key data points regarding trial numbers, phases, therapy types, and approved products were extracted and analyzed descriptively by region.ResultsA cumulative total of 10,373 cell therapy clin

Schnitzler's Syndrome: A Diagnostic Consideration in Evaluating the Constellation of Monoclonal Gammopathy and Chronic Urticaria.

Schnitzler's syndrome is a rare clinical entity characterized by intermittent, non-pruritic urticarial rash, fevers, arthralgias, myalgias and monoclonal gammopathy, most commonly of the immunoglobulin M (IgM) subtype. Schnitzler's syndrome should be considered in the differential diagnosis of fever of unknown origin. We report a case of a 56-year-old healthy Caucasian female, who initially presented to the primary care physician's office with complaints of severe generalized fatigue and myalgias involving thighs and calves. Patient subsequently underwent extensive rheumatologic workup, and wa

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… Interestingly, it is noted that anakinra offsets the development of these lymphoproliferative disorders. AA amyloidosis can develop in untreated cases [ 2 , 3 , 6 , 8 ]. …