Lymphology最新文献

Lymphangioplasty is a technique of reconstructive lymphatic surgery where subcutaneous lymphatic neocollectors are created, using surgical threads, nanofibrillar collagen threads, plastic tubes or autologous tissue flaps. The history and success rates of these techniques are outlined and a classification for lymphangioplasty techniques is proposed. The use of absorbable surgical threads is suggested for modern attempts of thread lymphangioplasties. The results of such a thread lymphangioplasty should be compared with that of implanted nanofibrillar collagen threads or plastic tubes in order to evaluate whether the technique itself or the material used is responsible for the therapeutic success.

There is a lack of large epidemiological studies focusing on the prevalence of lymphedema. Vital registration data from the United States (US) (1999-2020) and Veneto, Italy (2008-2021) were analyzed. Lymphedema-related deaths were identified using disease-specific ICD-10 codes and served to estimate the burden of disease in the general population. We studied (i) the lymphedema-specific proportionate mortality as a proxy of the disease-specific prevalence, (ii) the prevalence of lymphedema in key patient subgroups, and (iii) age and sex-specific mortality rates. The prevalence of lymphedema increased over the last two decades with marked sex-specific differences: in the US, the estimated prevalence of lymphedema was 2.7 per 10,000 deaths for women and 1.5 per 10,000 deaths for men. In Veneto, the prevalence was 3.0 per 10,000 deaths for women and 1.1 per 10,000 deaths for men. The prevalence of lymphedema was 2- to 20-times in specific subgroups of patients, including those with obesity, skin infections, hypertension, diabetes mellitus, breast/gynecological cancers, and venous thromboembolism. The estimated prevalence of lymphedema is 2- to 3-times higher than previously thought and has been increasing for the past two decades. These results will serve as a reference for future research in this field.

Primary lymphedema (PLE) is a chronic disease caused by lymphatic dysplasia and progresses to irreversible tissue edema and hypertrophy. Understanding of PLE has been hitherto limited. The aim of this study is to devise an updated classification system for PLE of 1013 patients with PLE of lower limb were enrolled. Sex, age of onset, location, family history and morbidity were documented. The lymphatic imaging findings of magnetic reso-nance lymphography (MRL), indocyanine green lymphography (ICGL) and lymphoscin-tigraphy (LSG), skin tissue immunohisto-chemical staining, whole exome sequencing and the correlation of genotype-phenotype were evaluated. Patients were divided into a congenital onset category and a late onset category. The late onset category was further divided according to developmental age. The ratio of congenital-onset to late-onset PLE was 1:4 and that the highest incidence was in adolescence. The sex ratio was 1.04:1 and 1.5:1 in congenital-onset and late-onset groups, respectively. Three major lymphatic anomalies were identified, in which segmental lymphatic dysfunction, characterized by delayed or partial demonstration of lymph vessels, is the most common and associated with FLT4, GJC2, CELSR1, and PTPN14 mutations. The next most common type is lymphatic hyperplasia, which is associated with FOXC2 and GATA2 variants, followed by initial lymphatic aplasia or dysfunction, which is more common in pa-tients with congenital PLE and associated with FLT4 mutation. A functional and structural combined classification of lymphatic anomalies is proposed, which includes segmental lymphatic dysfunction, lymphatic hyperplasia and initial lymphatic aplasia or dysfunction.

This study assesses the impact of an advanced intermittent pneumatic compression device (IPC - Lympha Press® Optimal Plus) when added to Complete Decongestive Therapy (CDT) compared to CDT alone on volume reduction of limbs with lymphedema. The goal is to maximally reduce edema in preparation for microsurgery. Fifty subjects scheduled for Multiple Lymphatic-Venous Anastomosis (MLVA) were randomly (sequentially) assigned to experimental or control group: 25 (21 females and 4 males) in the experimental IPC group and 25 (20 females and 5 males) in the control group. The two groups were similar in age, sex distribution, and type of lymphedema. Results indicate the IPC group reported greater volume loss than the control group (p= 0.00137) comparing final vs. initial limb volume. The average percentage edema volume loss achieved with added IPC was two times greater (11.7%) than in the control group (5.0%). When differences in treatment duration were accounted for, the IPC group achieved consistently greater proportional volume loss (12.83% vs 6.30%) than conservative therapy alone. In our pilot study, IPC added to CDT resulted in greater proportional volume loss and provides better preparation for MLVA surgery.

The technique of lymphangioplasty or capillary thread drainage was historically performed with subcutaneously implanted surgical threads. It has recently been revived by introducing a thread-like aligned nanofibrillar collagen matrix (BioBridge^tm). These collagen threads consitute subcutaneous neocollectors along which guided lymphangiogenesis is said to occur secondarily. We present for the first time a tissue examination of a 10-month implanted BioBridge^tm sample with surrounding tissue from a human subject by histology, scanning and transmission electron microscopy.

Primary lymphedema of the foot and toes could be sometimes misdiagnosed by lymphoscintigraphy as a whole lower limb lymphatic insufficiency (LLLI). This is caused by using standard lymphoscintigraphic protocol based on one interstitial injection of radiotracer applied into the first interdigital space followed by image analysis of lower limb lymphatic vessels and lymph nodes. Here, we show that a modification of the lymphoscintigraphic protocol and introduction of a second dose of radiotracer right above the inner ankle to the clinically healthy tissue can more accurately describe morphological abnormalities of the superficial lymphatic system at the lower limb and thereby refine the diagnosis of the LLLI. Fourteen patients with swelling of the foot and toes (16 lower limbs) were examined using standard lymphoscintigraphic protocol. Subsequently, modified lymphoscintigraphy was performed. While standard lymphoscintigraphy showed severe lymphatic insufficiency of the superficial lymphatic system in all 14 patients (in 16 lower limbs), including significantly reduced number of inguinal nodes, modified lymphoscintigraphy revealed almost normal morphology of superficial lymphatic vessels in 11 patients (in 13 lower limbs) throughout the entire lower limb proximal to the application site. In conclusion, using the modified lymphoscintigraphy protocol in patients with foot and toes primary lymphedema can refine diagnosis and follow-up medical management.

The proposed term "oncolymphology" encompasses the intimate relationship between cancer growth and the immune responses.

There are very limited studies on the relationship of inguinal lymph node number and volume correlated with lower extremity lymphedema severity. In this IRB-approved retrospective study, patients who obtained an MRI for lower extremity lymphedema and who did not have lymph node resection or biopsy were identified. The MRI images were used to determine the number and volume of inguinal lymph nodes for each limb in addition to fat and fluid-based scoring using a validated grading system. Wilcoxon signed-rank tests were used to compare the greater-affected limbs with the lesser-affected limbs. The spear-man-rank correlation was performed on a 'per limb' basis for MRI-based scoring and clinical parameters with ipsilateral lymph node number and volume and for differences between the limbs. A total of 32 patients were included. The greater-affected limb had higher MRI fluid scores (median (interquartile range) = 3 (3 - 3) vs. 0 (0 - 1), (p < 0.01) relative to the contra-lateral limb and had a median fat asymmetry score of 2 (1 - 3). On the per-limb analysis, lymph node number and volume inversely correlated with total MRI scores (ρ = -0.47, p < 0.01 for node number and volume). The difference of lymph node number and volume correlated with MRI score difference (node number: ρ = -0.66, p < 0.01; node volume: ρ = -0.64, p < 0.01) and perometer difference (node number: ρ = -0.58, p < 0.01; node volume: ρ = -0.59, p < 0.01). Inguinal lymph node number and volume inversely correlate with lower extremity edema presence and severity.

We have studied the lymphatic phenotypes of 2 mutations, known to cause abnormalities of lymphatics in humans, in mice. The Cx47 R260C mutation (variably penetrant in humans heterozygous for it and causing limb lymphedema) had an adult mouse phenotype of hyperplasia and increased lymph nodes only in homozygous condition but we did not find any anatomical phenotype in day 16.5 homozygous embryos. Mice harboring the Sos1 mutation E846K (causing Noonan's in man which occasionally shows lymphatic dysplasia) had no adult heterozygous phenotype in lymphatic vessel appearance and drainage (homozygotes are early embryonic lethals) while day 16.5 heterozygous embryos also had no detectable anatomical phenotype.