Lymphology最新文献

Covid-19 has physical damage as well as serious impact on the mental health in the community. Symptoms such as anxiety, depression, fear, stress, and sleep problems were more commonly reported during Covid-19 pandemic. The aim of this study was to assess the health status, psychological conditions, quality of life, and possible risk factors of patients with lymphedema during the pandemic. The study included male and female patients aged ≥18 years with primary or secondary upper or lower extremity lymphedema (stage 1, 2, or 3) who were followed in our outpatient clinic. The patients were interviewed by phone. Health and social status were examined using a questionnaire, Covid-19 phobia was assessed using Covid-19 Phobia Scale (C19P-S), and quality of life was assessed using Lymphedema Quality of Life Questionnaire Arm or Leg (LYMQOL). Anxiety and depression were evaluated using the Hospital Anxiety and Depression Scale (HADS). The HADS scores showed that 35% of the patients had severe risk for depression and 10% had severe risk for anxiety. Factors with negative effect on HADS were lower education level, sedentary lifestyle, failure to perform lymphedema exercises, weight gain, and lymphedema duration. The C19P-S scores were higher indicating greater phobia in the overall score and subscores in patients with primary lymphedema and secondary lymphedema without malignancy, younger patients, those who are not able to walk regularly, and those who are not able to perform self manual lymphatic drainage (self-MLD). Factors with negative effects on LYMQOL were stage 3 lymphedema, female gender, younger age, and longer disease duration. Patients who performed regular self-MLD and lymphedema exercises demonstrated positive effects on LYMQOL. The results of this study suggest that patients with lymphedema affected by the COVID-19 pandemic are mostly younger patients, individuals with primary lymphedema, individuals with non-malignant etiology, individuals who unable to perform regular walking, and those unable to perform self- MLD.

A recent genome-wide association study (GWAS) looking for the genes determining fingerprint and palmar crease patterns disclosed one gene, among many others, which causes lymphedema (CELSR1) while others influencing tissue growth. Since digital fluid influences the height of the volar pads, influences of lymphedema on dermatoglyphics should be sought.

An "incurable" lymphologist reflects on his life, unanswered questions, patients who became friends, and lessons learned during a brief encounter with a lymphatic soul-mate.

Diagnosing malignant lymphedema is a challenge in daily clinical practice. Clinically, patients may show clear signs of malignancy, but this is not always the case, and at times the diagnosis is not straightforward. In some patients, pain, hardness of the tissues, joint stiffness, proximal involvement, collateral circulation, or an acute onset will provide the clue to determining malignancy. Our aim is to describe several diverse scenarios of Secondary Malignant Lymphedema (SML) with the etiopathogenesis. One possible cause is lymphatic obstruction due to extrinsic compression of lymphatic vessels and/or nodes by either the primary tumor or metastatic masses. Lymphatic obstruction can also be caused by tumoral infiltration. This infiltration can affect both deep and regional nodes as well as cutaneous and subcutaneous vessels and is commonly known as lymphangitis carcinomatosa. Malignant lymphedema can also be secondary to obstruction of the venous flow due to tumoral venous thromboembolism or to extrinsic compression of the veins by tumors or adenopathic masses. Nevertheless, the most frequent cause of this illness is a mixed mechanism of compression of the lymphatic and venous systems. Frequently, SML is the first manifestation of relapse. When lymphedema appears abruptly, is progressive, with intense pain, associated with collateral circulation, or with hard and infiltrated skin or joint stiffness, SML must be ruled out with an urgent referral to the oncologist and an imaging evaluation.

Vascular malformations encompass a wide range of complex vascular lesions. Due to the extreme variability of clinical presentation, classification and their related syndromes presents a challenge. Here we describe a case of a boy presenting with Marfanoid habitus, cutaneous vascular malformations, and severe acute anemia due to ileal venous malformations. Although a panel of genetic markers for the Marfan phenotype was negative, we identified a de novo mutation in the TEK gene in the patient. This case supports expansion of the phenotypic spectrum of TEK-related vascular malformations.

Lymphedema is a debilitating disease characterized by abnormal lymphatic drainage, either due to primary maldevelopment of the lymphatic system or to secondary injury. The clinical features of primary and secondary lymphedema differ, with primary lymphedema more often involving progressive bilateral lower extremity disease as compared to secondary lymphedema characteristically having more localized symptoms related to the origin of injury. This case presentation describes a patient who presented with bilateral lower extremity swelling, left greater than the right, with imaging results to support the diagnosis of lymphedema. During the time he was followed in our clinic, our team witnessed rapid progression of his lymphedema despite compliance with conservative management. We believe that the primary mechanism of systemic damage to our patient's lymphatic system is the lenalidomide and bortezomib therapy prescribed to treat multiple myeloma. This review explores the relationship between lenalidomide, bortezomib, and lymphedema in efforts of understanding this unique pathology of iatrogenic lymphedema mimicking primary nature.

Although cancer survivors are recommended to exercise, they may lack confidence (self-efficacy) to be active. This research aimed to measure exercise barriers and related selfefficacy in individuals with cancer-related lymphedema as well as examine relationships between self-efficacy and participant characteristics. A cross-sectional survey was undertaken in individuals with cancer-related lymphedema using a validated 14-item Likert scale assessing self-efficacy to overcome general and lymphedema-specific exercise barriers (0%=not at all confident, 100%=extremely confident). Demographic, medical and lymphedema data were also collected. Of 109 participants (52% response), 79% (n=86) had breast cancer-related lymphedema. Participants were found to be moderately confident to exercise when facing general (48% [95% CI: 44, 52]) and lymphedema- specific exercise barriers (51% [95% CI: 47, 55]). Participants who were female, sedentary (p<0.05), had lymphedema for ≥2 years, and reported greater symptom burden (p<0.05) recorded lower general exercise barriers selfefficacy. Lower lymphedema-specific exercise barriers self-efficacy was reported by individuals who were sedentary, had cancers other than breast, and higher symptom burden. These findings suggest general and lymphedema- specific barriers challenge exercise confidence in those with cancer-related lymphedema, and strategies tailored to improve confidence in overcoming exercise barriers are warranted. Supporting individuals to be sufficiently active during and following cancer treatment should consider behavior change strategies tailored to the unique needs faced by individuals with lymphedema.

Milroy disease, known as primary congenital lymphedema, is characterized by chronic tissue swelling due to impaired lymphatic drainage and is inherited in an autosomal dominant manner. This study reports a rare case of Milroy disease affecting siblings from unaffected parents. A one-month-old female infant presented with swelling of the bilateral calf and the dorsum of the feet which had been present since birth. Her 14-month-old brother had a similar presentation since birth with swelling of the bilateral calf and the dorsum of the feet. Milroy disease was diagnosed based on the clinical findings of bilateral lower limb swelling and confirmed by molecular genetic testing. The patient and her family, including her brother, parents, and maternal grandfather, were genetically tested, and two novel missense mutations (NM_182925.4: c.2534T>C; p.Leu845Pro, c.4006G>A; p.Glu1336Lys) were found in the Fms-related tyrosine kinase (FLT4) gene. Mutations segregated by the parents who carried each mutation in the heterozygous state were identified in the patient and her brother. The present case report in which Milroy disease developed in all offspring of parents with a normal phenotype suggests the possibility of a compound heterozygous mutation or non-penetrance during the process of inheritance of Milroy disease.

The purpose of this study was to lymphoscintigraphically assess the effect of skin mobilization, nonspecific massage, and manual lymphatic drainage (MLD) on the root of the lower limb in patients with lower limb lymphedema. Lower limb root lymphoscintigraphical exams of 80 patients with lower limb lymphedema were analyzed. All patients underwent our stand 3 phase protocol and then were subjected to the 4th phase which included 3 subphases. Images were taken directly after the injection (subphase 1), after pinching and stretching the injection site (subphase 2), after nonspecific massage was applied to the injected site (subphase 3) and after manual lymphatic drainage of the injected site (subphase 4). The number of opened lymphatic pathways was analyzed and compared after and between each subphase (SP). SP 1 displayed open lymphatic pathways in 22 of the 80 cases (27.5%). SP 2 displayed newly opened lymphatic pathways in 48 of the 80 cases (60.0%). SP 3 displayed newly opened lymphatic pathways in 57 of the 80 cases (71.3%). Only 9 of these 57 cases did not show improvement following the next SP. SP 4 displayed newly opened lymphatic pathways in 60 of the 80 cases (75.1%). MLD improved the visualization of the lymphatic pathways in 48 cases (60%) compared to phase 3. MLD was the only technique to allow visualization of the lymphatic drainage at the level of the root of the edematous limb in 6 cases (7.5%). Physical therapy leads to a greater number of lymphatic collaterals opening in a region where no other complex decongestive therapy technique can be applied.

Thoracic duct drainage (TDD) is gaining renewed interest, largely due to accumulation of evidence supporting the gut-lymph model, where toxic mesenteric lymph from the intestine contributes to development of multi-organ failure in acute and critical illness (ACI). Advances in minimally invasive TDD have added to this growing interest. The English TDD literature has been previously reviewed, but the more extensive Eastern European literature has not been available to English readers. Therefore, we undertook a systematic search of Eastern European human TDD studies using Scopus and PubMed databases and Russian language websites. Indications for TDD, clinical outcomes, and complications were reviewed. 113 studies, published between 1965 and 2015, were reviewed. The most common indications for TDD were hepatic failure, acute pancreatitis, and peritonitis. It was often used late and when other treatment options had been exhausted. Human TDD appeared safe and probably effective, especially when combined with lymphosorption. The benefit appeared to correlate with the volume of lymph drained. A randomized controlled trial (and some case-control studies) showed reduced mortality in patients with ACI with TDD. Other benefits included rapid normalization of blood parameters and decreased organ edema. This review provides further support for the gut-lymph model and justification for high quality randomized controlled trials of TDD in ACI. It also highlights other potential indications for TDD, such as bridging patients with liver failure to surgery or transplant.