Lymphology最新文献

The purpose of this study was to compare plasma vitamin D levels in patients with breast cancer-related lymphedema (BCRL), breast cancer (BC) without lymphedema, and healthy populations, as well as relationship between clinical variables, lymphedema severity grading, and circulating vitamin D concentrations. This Case-control study was conducted between June 2017 and January 2025. The study comprised three cohorts: a case cohort of individuals diagnosed with breast cancer-related lymphedema (BCRL), a disease-matched control cohort of breast cancer (BC) patients without lymphedema (demographically aligned with the BCRL cohort by age and sex), and a reference cohort of healthy participants matched by age and sex. A total of 603 female individuals were included in the study with 201 individuals in each group. Post-hoc Games-Howell test analyses revealed a lower level of Vitamin D in the BCRL group compared to the BC and control groups (p<0.001, p<0.001) and lower levels in the BC group compared to the control group (p<0.001). Vitamin D deficiency was statistically significantly more frequent in BCRL group than in BC and control groups (p:<0.001) and the proportion of those with normal vitamin D were statistically significantly more frequent in control group than in BCRL and BC and control groups (p:<0.001). Analysis revealed a strong, significant negative correlation between circulating vitamin D concentrations and advancing lymphedema stage (r: -0.578, p:<0.001). These observations imply that hypovitaminosis D may contribute to breast cancer pathogenesis and modulate the risk of treatment-associated morbidities, particularly lymphedema progression. Therefore, it is recommended that individuals diagnosed with BC or at risk of lymphedema have their vitamin D levels checked regularly and take supplements if necessary.

Lymphedema is a chronic, progressive impairment of the lymphatic system that can impact activities and quality of life. Research regarding conservative management of lymphedema primarily consists of complete decongestive therapy (CDT) and compression devices intended to promote lymphatic and venous return. Advanced pneumatic compression devices (APCDs) contain multiple programmable sleeves designed to mimic manual lymph drainage at home. This study aims to determine the effectiveness of APCDs in the treatment of lymphedema through the completion of a systematic review and meta-analysis. A systematic search of five databases was conducted, spanning the years 2010-2021. Only experimental designs of moderate or strong quality were chosen and final review set consists of twelve articles. APCDs are found to be effective for lymphedema treatment based on differences in pre- and post-intervention limb volumes and patient-reported outcomes. Two meta-analyses evaluated the impact of APCDs on both rates of cellulitis and manual therapy. Both analyses demonstrated significant decreases in rates after the APCD interventions. The data showed consistent reduction of limb volume and improved patient-reported outcomes, indicating that APCDs are generally effective as a complementary intervention to CDT in the maintenance phase of lymphedema treatment.

The Ketogenic Diet (KD) is currently the most widely studied nutritional approach for patients with lipedema, although its use may cause important drawbacks especially in case of comorbidities. A Modified Mediterranean Diet (MMed) is more easily adaptable, better tolerated, and can be temporarily replaced by a ketogenic diet for a short period of time if necessary using a sort of "sandwich" approach. We report 10 clinical cases with lipedema and comorbidities subjected to a hypocaloric MMed for 6 months. All patients were properly assessed by anthropometric measurements and body composition before and at the end of nutritional treatment. Only 2 patients needed a short period of KD due to poor response to MMed. At the end of 6-month follow-up, patients showed weight loss with reduction in body circumferences and improvement in body composition. In the two cases where it was necessary to resort to a period of KD, good results were achieved allowing a return to the MMed for the maintenance. In patients suffering from lipedema with associated comorbidities and concomitant pharmacological treatments, the use of MMed as first line treatment allows achievement of better metabolic balance, greater compliance, and improved body composition.

In Memoriam: Leo Clodius. April 29, 1930 - September 17, 2025.

This report describes the technique of lymphopseudoaneurysm (LPA) detection by pressurized contrast injection into a lymphocele, followed by image-guided embolization. A 37-year-old man with pancreatic neuro-endocrine tumor, portal cavernoma, and liver metastasis underwent pancreaticoduodenectomy, right hepatectomy, lymphadenectomy, and left portal vein reconstruction. Postoperative course was complicated by refractory chylous ascites. Computed Tomography (CT) revealed a centrally located lymphocele in the abdominal cavity. Direct puncture of the lymphocele, followed by pressurized contrast injection revealed a tubular connection to a smaller retroperitoneal collection near the surgical site, which was considered to be the culprit LPA. After catheter directed embolization of this LPA with N-butyl-cyanoacrylate glue, chylous ascites ceased and abdominal drainage catheters were removed 12 days later.

Kikuchi-Fujimoto disease (KFD) shares similar clinical manifestations and ultrasonic features with non-specific reactive lymphadenopathy (NSRL). This study aims to elucidate the independent predictors distinguishing these two diseases. A total of 136 lymph nodes (136 patients) with pathologically proven KFD from January 2015 to December 2019 were included. The control group comprised 117 NSRL lymph nodes (117 patients). Clinical information and imaging features were collected and analyzed. The main complaints of KFD were fever (84.6%). The patients with KFD and immune system diseases were older and had longer duration of symptoms. No significant differences (p< 0.05) were observed in sex, white blood cell count, short and long axis, shape, and margin. A binary logistic regression analysis revealed that the independent diagnostic factors were patient age (odds ratio [OR]=3.120, p=0.008), fever or pain (OR=0.100, p=0.038), failure of empirical antibiotic or antiviral therapy (OR= 305.88, p<0.001), vascular pattern (OR=0.049, p=0.042), CRP (OR=2.679, p=0.035), laterality (OR=0.352, p=0.020), and loose conglomeration (OR=3.605, p=0.019). The combined diagnosis effectiveness was 88.9%. Fever/pain, age range of 20-32 years, increased CRP, ineffective empirical treatment, loose conglomeration, unilateral, and non-abnormal vascular pattern were independent predictors.

There were multiple formatting issues identified with the article: "The Re-Discovery of Dural (Meningeal) Lymphatics: Amnesia or Ambition?" By R.P. Erickson (Lymphology 56 (2023) 125-130; https://doi.org/10.2458/lymph.6179). These have been corrected and the article has been updated. The journal regrets the original errors in formatting.

Lymphedema is a chronic inflammatory disease caused by defective lymphatic system function or development that proceeds in ab-normal fluid drainage and edema of the peripheral tissues. This retrospective study evaluates the potential role of transcription factors in the development of primary lymphedema. We analyzed 408 Italian primary lymphedema patients for pathogenic variants in genes related to the transcription factor network. Eighteen likely pathogenic genetic variants were identified in 5 genes - FOXC1, FOXC2, NOTCH1, RORC, and SOX18 - most of which have not been previously reported in the literature. Although majority of these genetic variants resulted in premature termination of the proteins, a single missense variant was identified in FOXC2 and analyzed in this study using molecular modeling approaches to evaluate its structural changes. These results suggested possible deleterious effects of the analyzed variant and supported its potential pathogenicity. Our findings provide additional evidence that associates variants in transcription factors to the onset of lymphedema and support the addition of the candidate genes FOXC1, NOTCH1, and RORC in primary lymphedema diagnostic practice.

Central lymphatic disorders of the lung have not received intense investigation. Lymphatic system physiology is presented in the context of historical developments and basic lung lymphatic anatomy is reviewed followed by emerging characteristics of primary and secondary pathophysiological disturbances of lymphatic involvement in a number of pulmonary diseases including Gorham-Stout disease, pulmonary edema and infections and inflammatory conditions including lymphangioleiomyomatosis (LAM). The future includes potential molecular targeting of lymphangiogenesis or lymphatic vessels for interventional occlusion. This article is an amalgamation of presentations at the 2023 ISL International Congress of Lymphology, Genoa, Italy in a special symposium on central and regional lymphatic system in health and disease and as part of a Special Symposium on the Lymphatic system of the Heart and Lung in Health and Disease at the 26th International Congress of Lymphology meeting held in Barcelona, Spain, September 2017, which has been updated to 2024.

Lymphatic anatomy of the abdomen is reviewed with focus on hepatic and mesenteric vessels in normal and pathologic conditions. Anatomy and pathophysiology is highlight using both specialized fluoroscopy and T2 Dynamic Contrast MR lymphangiography (DCMRL). Plastic bronchitis, chylothorax, protein losing enteropathy, and both cirrhosis and ascites due to hepatic lymphatics are highlighted. Presented at the 2023 ISL International Congress of Lymphology, Genoa, Italy in a special symposium on central and regional lymphatic system in health and disease.