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Medicina (Kaunas, Lithuania) Nov 2023Lymphedema is a progressive condition. Its therapy aims to reduce edema, prevent its progression, and provide psychosocial aid. Nonsurgical treatment in advanced stages... (Review)
Review
Lymphedema is a progressive condition. Its therapy aims to reduce edema, prevent its progression, and provide psychosocial aid. Nonsurgical treatment in advanced stages is mostly insufficient. Therefore-in many cases-surgical procedures, such as to restore lymph flow or excise lymphedema tissues, are the only ways to improve patients' quality of life. Lymphoscintigraphy (LS), near-infrared fluorescent (NIRF) imaging-also termed indocyanine green (ICG) lymphography (ICG-L)-ultrasonography (US), magnetic resonance lymphangiography (MRL), computed tomography (CT), photoacoustic imaging (PAI), and optical coherence tomography (OCT) are standardized techniques, which can be utilized in lymphedema diagnosis, staging, treatment, and follow-up. The combined use of these imaging modalities and self-assessment questionnaires deliver objective parameters for choosing the most suitable surgical therapy and achieving the best possible postoperative outcome.
Topics: Humans; Quality of Life; Indocyanine Green; Coloring Agents; Lymphedema; Lymphography
PubMed: 38004065
DOI: 10.3390/medicina59112016 -
Cancer Biology & Medicine Dec 2023
Topics: Hodgkin Disease; Humans; Antineoplastic Combined Chemotherapy Protocols
PubMed: 38164723
DOI: 10.20892/j.issn.2095-3941.2023.0427 -
International Journal of Molecular... Jun 2023Sarcoidosis is a multisystemic disease of unknown etiology characterized by the formation of granulomas in various organs, especially lung and mediastinal hilar lymph... (Review)
Review
Sarcoidosis is a multisystemic disease of unknown etiology characterized by the formation of granulomas in various organs, especially lung and mediastinal hilar lymph nodes. The clinical course and manifestations are unpredictable: spontaneous remission can occur in approximately two thirds of patients; up to 20% of patients have chronic course of the lung disease (called advanced pulmonary sarcoidosis, APS) resulting in progressive loss of lung function, sometimes life-threatening that can lead to respiratory failure and death. The immunopathology mechanism leading from granuloma formation to the fibrosis in APS still remains elusive. Recent studies have provided new insights into the genetic factors and immune components involved in the clinical manifestation of the disease. In this review we aim to summarize the clinical-prognostic characteristics and molecular pathways which are believed to be associated with the development of APS.
Topics: Humans; Pulmonary Fibrosis; Sarcoidosis; Sarcoidosis, Pulmonary; Lung; Granuloma
PubMed: 37445947
DOI: 10.3390/ijms241310767 -
Molecular Imaging and Biology Feb 2024Study of the lymphatic system, compared to that of the other body systems, has been historically neglected. While scientists and clinicians have, in recent decades,... (Review)
Review
Study of the lymphatic system, compared to that of the other body systems, has been historically neglected. While scientists and clinicians have, in recent decades, gained a better appreciation of the functionality of the lymphatics as well as their role in associated diseases (and consequently investigated these topics further in their experimental work), there is still much left to be understood of the lymphatic system. In this review article, we discuss the role lymphatic imaging techniques have played in this recent series of advancements and how new imaging techniques can help bolster this wave of discovery. We specifically highlight the use of lymphatic imaging techniques in understanding the fundamental anatomy and physiology of the lymphatic system; investigating the development of lymphatic vasculature (using techniques such as intravital microscopy); diagnosing, staging, and treating lymphedema and cancer; and its role in other disease states.
Topics: Humans; Lymphatic System; Lymphedema; Diagnostic Imaging; Neoplasms; Disease Progression; Lymph Nodes
PubMed: 37195396
DOI: 10.1007/s11307-023-01827-4 -
Blood Reviews Mar 2024Idiopathic multicentric Castleman disease (iMCD) is a rare disease, and it is likely underdiagnosed because of the heterogeneity of clinical manifestations and... (Review)
Review
Idiopathic multicentric Castleman disease (iMCD) is a rare disease, and it is likely underdiagnosed because of the heterogeneity of clinical manifestations and laboratory findings. While the disease leads to significant morbidity and mortality, its causes are not yet fully elucidated. There have been significant advances in diagnosis and treatment of iMCD in the past decade, including the approval of the anti-IL-6 antibody siltuximab. In this review, we provide an update of the many new developments and publications surrounding iMCD.
Topics: Humans; Castleman Disease
PubMed: 38087716
DOI: 10.1016/j.blre.2023.101161 -
The Journal of Clinical Investigation Mar 2024The lymphatic vascular system is gaining recognition for its multifaceted role and broad pathological significance. Once perceived as a mere conduit for interstitial... (Review)
Review
The lymphatic vascular system is gaining recognition for its multifaceted role and broad pathological significance. Once perceived as a mere conduit for interstitial fluid and immune cell transport, recent research has unveiled its active involvement in critical physiological processes and common diseases, including inflammation, autoimmune diseases, and atherosclerosis. Consequently, abnormal development or functionality of lymphatic vessels can result in serious health complications. Here, we discuss lymphatic malformations (LMs), which are localized lesions that manifest as fluid-filled cysts or extensive infiltrative lymphatic vessel overgrowth, often associated with debilitating, even life-threatening, consequences. Genetic causes of LMs have been uncovered, and several promising drug-based therapies are currently under investigation and will be discussed.
Topics: Humans; Lymphatic Abnormalities; Lymphatic Vessels; Lymphatic System
PubMed: 38488007
DOI: 10.1172/JCI172844 -
Frontiers in Cardiovascular Medicine 2024Lymphatic endothelial cells (LECs) line lymphatic vessels, which play an important role in the transport of lymph fluid throughout the human body. An organized lymphatic... (Review)
Review
Lymphatic endothelial cells (LECs) line lymphatic vessels, which play an important role in the transport of lymph fluid throughout the human body. An organized lymphatic network develops via a process termed "lymphangiogenesis." During development, LECs respond to growth factor signaling to initiate the formation of a primary lymphatic vascular network. These LECs display a unique metabolic profile, preferring to undergo glycolysis even in the presence of oxygen. In addition to their reliance on glycolysis, LECs utilize other metabolic pathways such as fatty acid β-oxidation, ketone body oxidation, mitochondrial respiration, and lipid droplet autophagy to support lymphangiogenesis. This review summarizes the current understanding of metabolic regulation of lymphangiogenesis. Moreover, it highlights how LEC metabolism is implicated in various pathological conditions.
PubMed: 38798921
DOI: 10.3389/fcvm.2024.1392816 -
World Journal of Gastroenterology Jun 2023Follicular lymphoma (FL) is the most common low-grade lymphoma, and although nodal FL is highly responsive to treatment, the majority of patients relapse repeatedly, and...
Follicular lymphoma (FL) is the most common low-grade lymphoma, and although nodal FL is highly responsive to treatment, the majority of patients relapse repeatedly, and the disease has been incurable with a poor prognosis. However, primary FL of the gastrointestinal tract has been increasingly detected in Japan, especially due to recent advances in small bowel endoscopy and increased opportunities for endoscopic examinations and endoscopic diagnosis. However, many cases are detected at an early stage, and the prognosis is good in many cases. In contrast, in Europe and the United States, gastrointestinal FL has long been considered to be present in 12%-24% of Stage-IV patients, and the number of advanced gastrointestinal cases is expected to increase. This editorial provides an overview of the recent therapeutic advances in nodal FL, including antibody-targeted therapy, bispecific antibody therapy, epigenetic modulation, and chimeric antigen receptor T-cell therapy, and reviews the latest therapeutic manuscripts published in the past year. Based on an understanding of the therapeutic advances in nodal FL, we also discuss future possibilities for gastroenterologists to treat gastrointestinal FL, especially in advanced cases.
Topics: Humans; Lymphoma, Follicular; Neoplasm Recurrence, Local; Gastrointestinal Neoplasms; Prognosis; Transforming Growth Factor beta
PubMed: 37398889
DOI: 10.3748/wjg.v29.i23.3574 -
International Journal of Infectious... Mar 2024
Topics: Humans; Onchocerciasis; Elephantiasis, Filarial; Africa; Disease Eradication; Neglected Diseases
PubMed: 38280664
DOI: 10.1016/j.ijid.2024.01.016 -
Blood Advances Jul 2023The genetic mechanisms associated with splenic marginal zone lymphoma (SMZL) transformation are not well defined. We studied 41 patients with SMZL that eventually...
The genetic mechanisms associated with splenic marginal zone lymphoma (SMZL) transformation are not well defined. We studied 41 patients with SMZL that eventually underwent large B-cell lymphoma transformation. Tumor material was obtained either only at diagnosis (9 patients), at diagnosis and transformation (18 patients), and only at transformation (14 patients). Samples were categorized in 2 groups: (1) at diagnosis (SMZL, n = 27 samples), and (2) at transformation (SMZL-T, n = 32 samples). Using copy number arrays and a next-generation sequencing custom panel, we identified that the main genomic alterations in SMZL-T involved TNFAIP3, KMT2D, TP53, ARID1A, KLF2, 1q gains, and losses of 9p21.3 (CDKN2A/B) and 7q31-q32. Compared with SMZL, SMZL-T had higher genomic complexity, and higher incidence of TNFAIP3 and TP53 alterations, 9p21.3 (CDKN2A/B) losses, and 6p gains. SMZL and SMZL-T clones arose by divergent evolution from a common altered precursor cell that acquired different genetic alterations in virtually all evaluable cases (92%, 12 of 13 cases). Using whole-genome sequencing of diagnostic and transformation samples in 1 patient, we observed that the SMZL-T sample carried more genomic aberrations than the diagnostic sample, identified a translocation t(14;19)(q32;q13) present in both samples, and detected a focal B2M deletion due to chromothripsis acquired at transformation. Survival analysis showed that KLF2 mutations, complex karyotype, and International Prognostic Index score at transformation were predictive of a shorter survival from transformation (P = .001; P = .042; and P = .007; respectively). In summary, SMZL-T are characterized by higher genomic complexity than SMZL, and characteristic genomic alterations that could represent key players in the transformation event.
Topics: Humans; Splenic Neoplasms; Mutation; Translocation, Genetic; Lymphoma, Large B-Cell, Diffuse; Leukemia, Lymphocytic, Chronic, B-Cell
PubMed: 36995085
DOI: 10.1182/bloodadvances.2022009415