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PubMed1981

[Diffuse cystic angiomatosis of bone. A report on one case followed-up over five years (author's transl)].

The authors report a case of diffuse cystic angiomatosis of bone. The follow-up over five years showed a dramatic worsening which has seldom been observed. The roentgenographic aspect of this rare disease seems always similar, but non specific. The pathologic diagnosis is difficult. The bony involvement is due to a widespread vascular dysplasia, and the final diagnosis relies upon the association with other arteriovenous or lymphatic disorders, or with an abnormal lymphogram.

Angiomatosis↗
PubMed1996

Management of thoracic duct complex lesions (chylothorax): experience in 16 patients.

From our experience in 16 patients with persistent chylothorax from fistulas of the thoracic duct or its tributaries, we conclude that no standard treatment is uniformly successful and multimodality therapy should be considered. In selected patients, an anastomosis between ectatic lymphatics or hyperplastic lymph nodes and an adjacent vein may be attempted. Chylothorax from "leakage" of the thoracic duct or its tributaries is rare. Rupture of the thoracic duct superior to the sixth thoracic vertebrae generally results in a left-sided chylothorax; below that level, injury usually results in a right-sided chylothorax. The etiology is heterogeneous and includes blunt trauma, penetrating wounds (1), iatrogenic operative injury and lymphatic obstructions due to congenital abnormalities, inflammatory processes or neoplasms. Based on our experience in 16 patients with persistent chylothorax from thoracic duct complex lesions, we review the available treatment options.

Adolescent↗
PubMed2002

Research perspectives in inherited lymphatic disease.

The hereditary lymphedemas provide an opportunity to identify genes involved in normal and deranged lymphatic development. Genetic analysis of families with Milroy's disease identified mutations in VEGFR3 as a cause of congenital lymphedema, confirming the importance of VEGFC/VEGFR3 signaling in lymphatic development. These observations led to the identification of a mouse model for primary lymphedema, and subsequent analysis of this mouse model, using transgenic and gene transfer techniques, has provided initial clues to the development of a biologically based therapy for primary lymphedema. Of more importance from a public health perspective is the fact that manipulation of this pathway may lead to effective therapies for the more prevalent forms of secondary lymphedema. Identification of FOXC2 as the gene mutated in the lymphedema-distichiasis syndrome has revealed new molecular insight into lymphatic development. Molecular analysis of the FOXC2 pathway may provide clues to developmental pathways shared by the lymphatic system and the other developmental abnormalities associated with this complex syndrome. With improving knowledge of the human genome, genetic analysis of families with lymphedema continues to offer one of the most promising approaches to identifying genes influencing lymphatic development.

DNA-Binding Proteins↗
PubMed2003

FOXC2 haploinsufficient mice are a model for human autosomal dominant lymphedema-distichiasis syndrome.

Lymphedema-distichiasis (LD) (OMIM 153400) is a rare autosomal-dominant condition characterized by pubertal onset of lower limb lymphedema and an aberrant second row of eyelashes arising from the meibomian glands. In some patients cardiac, skeletal and other defects coexist. We previously identified inactivating, nonsense and frameshift mutations in the forkhead transcription factor FOXC2 in affected members of LD families. To further delineate the relationship of FOXC2 deficiency to the clinical (and lymphangiodysplastic) phenotype in this syndrome, we performed dynamic lymphatic imaging and immunohistochemical examination of lymphatic tissues in mice heterozygous (+/-) for a targeted disruption of Foxc2. Adult heterozygote mice characteristically exhibited a generalized lymphatic vessel and lymph node hyper plasia and rarely exhibited hindlimb swelling. Retrograde lymph flow through apparently incompetent interlymphangion valves into the mesenteric nodes, intestinal wall and liver was also observed. In addition, Foxc2 +/- mice uniformly displayed distichiasis. We conclude that Foxc2 haploinsufficient mice mimic closely the distinctive lymphatic and ocular phenotype of LD patients. Furthermore, the craniofacial, cardiovascular and skeletal abnormalities sometimes associated with LD have previously been shown to be fully penetrant in homozygous Foxc2 null mice. This Foxc2 mutant mouse thus provides an ideal model for exploring molecular mechanisms and physiologic events in mesenchymal differentiation associated with lymphatic growth and development and the clinical abnormalities seen in human LD syndrome.

Animals↗
PubMed1982

[Non-invasive diagnosis of isolated chylopericardium using precordial pericardial imaging after oral administration of 131I-triolein: report of a case].

Chylopericardium is a rare disease and affects both sexes equally from neonate to adult. Usually, there are abnormal connections between the pericardial cavity and thoracic lymphatic systems. These connections are detected by (1) recovery of orally administered Sudan III from pericardial fluid, (2) evidence of radioactivity in the pericardial fluid by paracentesis after oral administration of 131I-labeled triolein, and (3) lymphangiography. However, these method are technically difficult and invasive, thus sometimes dangerous for children. We employed precordial pericardial imaging after oral administration of 131I-labeled triolein on a 9-year-old Japanese girl with isolated chylopericardium before and after surgery. Abnormal connections and the back-ward flow to the pulmonary lymphatics were demonstrated by this method. This is an easy, non-invasive, reliable and safe method for detecting the abnormal connections of pericardial and lymphatic systems in children with chylopericardium.

Child↗
PubMed1976

Canine lung allograft lymphatic alterations.

Lymphatic obstruction has not been emphasized as a feature of lung allograft rejection. However, accumulation of fluid and cellular infiltrate, aggravated by lymph stasis, results in impaired lung function. In this study, lung specimens were recovered at varying times up to 133 days after either reimplantation (7 dogs) or allografting (29 dogs). Azathioprine and prednisone were administered to 17 allograft recipients. The presence of abnormally dilated perivascular, peribronchiolar, and subpleural lymphatic channels was a consistent histological finding, most striking in specimens recovered from untreated allograft recipient dogs. Attenuated lymphatic alterations were noted in immunosuppressed allograft recipients. In these animals the pulmonary lymphatics seemed to be ineffectual in clearing the allograft of the accumulating cellular infiltrates and fluid during rejection.

Animals↗
PubMed1987

[Classical Kaposi's sarcoma. Study of the lymphatic system of lower limbs].

Eight patients with classical Kaposi's sarcoma histologically confirmed underwent lymphoscopic and/or lymphographic exploration of the lower limbs where the lesions were located. In all patients lymphoscopy showed abnormal images ranging from irregular distribution of the colouring agent to "starlike images" and dermal reflux. Lymphography was performed in 3 patients and demonstrated, in 2 of them, "lymphatic pools", obvious hyperplasia of the superficial lymphatic vessels and areas of lymphangectasia. The finding in all 8 patients of dynamic abnormalities of the lymphatic system should encourage further investigations in this field, notably to detect a possible correlation between lymph stasis, disorders in regional immunity and the development of classical Kaposi's sarcoma.

Female↗
PubMed1996

Acute-phase plasma proteins in gastric cancer: association with metastatic potential and prognosis.

We evaluated the possibility that acute-phase plasma proteins such as alpha 1-antichymotrypsin (ACT) and immunosuppressive acidic protein (IAP) might be useful predictors of lymph node metastasis and prognosis in patients with gastric cancer. Both ACT and IAP levels generally increased according to the pTNM stage. Patients with both abnormal IAP and ACT levels showed a high risk of lymphatic and hepatic metastasis as well as peritoneal dissemination, with a resultant poor prognosis. Patients who had abnormal IAP levels with or without abnormal ACT levels had a significantly higher risk of lymph node metastasis, as well as more invasive tumors and a worse prognosis than those who had normal IAP levels with or without abnormal ACT levels. In combination group 4 [IAP(+) ACT(-) vs. IAP(-) ACT(+)] lymphatic metastasis was seen more often with isolated IAP(+) (76.4%) than with ACT(+) (52.9%) (p < 0.0045), especially in the subgroup of poorly differentiated adenocarcinoma (POR; p < 0.0177). However, this does not demonstrate that ACT(+) is a protective factor against lymphatic invasion, because the results of combination group 6 [IAP(-) ACT(+) vs. IAP(-) ACT(-)] show that isolated ACT(+) is also significantly related to lymphatic metastasis (p < 0.001). The same is true for the subgroup of signet ring cell carcinomas (p = 0.038), but it has not been tested versus the POR subgroup.

Acute-Phase Proteins↗
PubMed1995

Translocation (2;14)(p13;q32) in CD10+ ;CD13+ acute lymphatic leukemia.

The rare t(2;14)(p13;q32) was previously described in the three pediatric patients with acute lymphatic leukemia. In these cases this abnormality was found at diagnosis, manifested the sole chromosomal abnormality, and was associated with a favorable prognosis. We here describe three cases of leukemia where such translocations were found at relapse, were associated in two of the cases with additional known characteristic chromosomal aberration, and were associated with a grave prognosis. Interestingly enough, the malignant cells of all three patients shared the same surface antigens: CD34, HLA DR, CD10, CD20, and the myeloid marker CD13. The leukemic clone exhibiting t(2;14) probably evolved from a t(1;19)6q- pre-B acute lymphatic leukemia in one of the cases, and from a chronic phase Ph1 chromosome in another. The significance of the translocation and the coexistence of CD10 and CD13 on the same cell are discussed.

Adolescent↗
PubMed1986

Pulmonary edema.

The lungs are marvelously designed to handle fluid. The mechanical properties of the lungs and the lymphatics act simply and efficiently to drain fluid out of the pulmonary interstitium. Despite the enormous blood flow through the pulmonary and bronchial circulation, a dynamic equilibrium is maintained between fluid fluxing out of the vasculature into the pulmonary interstitium and fluid being drained out of the lungs by the lymphatics. This is obviously important because maintaining "dry" air spaces is essential for normal pulmonary function. Fluid accumulates in the lung when flux across the vascular endothelium exceeds lymphatic drainage. Two different types of abnormalities will result in accumulation of fluid, an increase in pulmonary microvascular pressure and an increase in the pulmonary vascular endothelial permeability to protein. Regardless of the type of abnormality causing pulmonary edema, fluid tends to accumulate in the lungs in a predictable pattern based on the same mechanical properties that normally keep the lung dry. Only by understanding the normal process of fluid handling will the clinician truly appreciate the consequences of pulmonary edema.

Aged↗
PubMed1991

Initial treatment of pulmonary edema: a physiological approach.

An understanding of the physiological principles involved in lung fluid balance is useful in the initial treatment of pulmonary edema. Normally, a very small volume of fluid is filtered from the pulmonary vasculature into the interstitial space. This interstitial fluid enters the pulmonary lymphatics and is transferred to mediastinal lymphatics at an estimated rate of 20 ml/hr. Under abnormal circumstances, fluid filtration may occur at such a rapid rate that it overwhelms the lymphatics and interstitial space and results in alveolar flooding. This may occur as a result of increased pulmonary vascular pressure or increased vascular permeability. The two general goals of initial therapy are (1) to relieve hypoxemia and (2) to reduce pulmonary capillary pressure. Relieving hypoxemia may require the use of supplemental oxygen by nasal prongs or mask, continuous positive airway pressure (CPAP) mask, or even endotracheal intubation and mechanical ventilation. Measures to decrease preload and thereby reduce pulmonary capillary pressure include sitting the patient up, administering a loop diuretic or morphine intravenously, and in some circumstances using sublingual nitroglycerin. After initial treatment is underway, a search for and specific management of the underlying cause of pulmonary edema can proceed.

Capillary Permeability↗
PubMed1982

[Morphological changes of venous and lymphatic vessels of dog extremities after ligation of major vessels].

Morphological alterations in venous and lymphatic vessels were studied on 38 dogs over time after ligation of the main veins of the hind limb and induction of experimental chronic venous failure. Under these conditions the operated limb developed marked lymphovenous congestion with ectasis and dilatation of blood and lymphatic vessels and capillaries, venous and lymphatic vessels of the limb followed by an increase in the number of the vessels due to high blood content of "reserve" capillaries and small veins in the presence of acute tissue edema. Then the limb developed compensatory-adaptive processes, namely reconstruction of the venous system accompanied by formation of collateral main veins and lymphatic collectors that partially compensated for abnormal venous blood flow. Prolonged impairment of hemocirculation and venous congestion led to anoxia of venous walls, sclerosis, dystrophic, inflammatory and focal sclerotic lesions in the adjacent tissue. This experimental model can be successfully used for solving the problems of the pathogenesis and therapy of chronic venous failure.

Animals↗
PubMed1987

Uremic pleuritis.

Pleural abnormalities of uremia have been recognized for many years but have been given little attention despite their high incidence. Mechanisms underlying pleural effusion relate to filtration forces across subpleural capillaries and lymphatic absorption, either of which can be abnormal in patients with renal failure. Uremic patients have increased susceptibility to many causes of pleural exudate. In addition, a specific uremic pleuritis has been characterized as necrotizing fibrinous sterile exudate that is often hemorrhagic. Spontaneous remission, often with recurrences, or constrictive pleural thickening requiring surgical decortication may occur. Neither the pathogenesis nor the appropriate treatment of uremic pleuritis has been established definitively.

Humans↗
PubMed2004

[Comparative analysis of lymphoscintigraphy between lipedema and lower limb lymphedema].

UNLABELLED: Lipedema is characterized by bilateral enlargement of the legs due to abnormal deposition of fat tissue from pelvis to ankles. It is seen most frequently in obese women. Lipedema appears to be a distinct clinical entity but may be confounded with lymphedema. AIM OF THE STUDY: To analyze and to compare between lipedema and lymphedema the qualitative and quantitative aspects of lymphoscintigraphy. METHODS: Fifteen women with lipedema were recruited. Mean age of onset of lipedema was 31.5 +/- 15 years. Body mass index was 35.1 +/- 7.9 kg/m2, 13 women were obese. Lipedema was compared to 15 cases of primary lymphedema (women: 13, men: 2) of the lower limbs (unilateral: 13, bilateral: 2), with a mean age at onset of 28.7 +/- 12.6 years. Lymphoscintigraphy of the lower limbs with morphologic (visualization of inguinal lymph nodes) and kinetic (half-life, lymphatic speed of the colloid) studies was performed in all cases. RESULTS: Absence of visualization of inguinal lymph nodes was observed in 14/15 cases of lymphedema and in 1/15 cases of lipedema (p<0.001). In the 13 cases of unilateral lymphedema, colloid half-life was higher in the pathologic limb than in the controlateral limb (230 +/- 92 vs 121 +/- 36 minutes, p<0.01) and lymphatic speed of the colloid was slower (6.91 +/- 0.86 vs 8.16 +/- 1.02 cm/min, p<0.001). The two patients with bilateral lymphedema had an increased half-life and decreased lymphatic speed of the colloid. Colloid half-life was significantly higher in lipedema than in controlateral limbs of lymphedema (154 +/- 23 vs 121 +/- 36 minutes, p<0.01) with no difference in lymphatic speed of the colloid. Colloid half-life was significantly higher in lymphedema than in lipedema (230 +/- 92 vs 154 +/- 23 minutes, p<0.01) and the lymphatic speed of the colloid was slower (6.91 +/- 0.86 vs 8.10 +/- 0.45 cm/min, p<0.001). CONCLUSION: Lower limb lymphoscintigraphy showed lymphatic insufficiency in lipedema without morphologic abnormality as seen in lymphedema. Lymphoscintigraphy is not indispensable but is a useful tool when diagnosis is doubtful. Treatment is difficult and may include weight loss and possible surgery.

Adipose Tissue↗
PubMed1981

Nonlymphoid hematopoietic malignant disease of the lymph nodes.

The extension of myeloproliferative malignant tumors to lymph nodes was studied in seven excision biopsies and 27 autopsies in which lymph nodes were obtained from all nodal regions. We observed the proliferation of poorly, partially, and well differentiated neoplastic cells with a predominance of immature cells of the granulocytic series and fewer cells of the erythroid and megakaryocytic cells lines. Disturbances of the lymph node architecture consisted of invasion by abnormal cells of the trabecular stroma, disrupting the reticulin mesh and extending toward the pericapsular area. In the lymph node a loose network of thin and thick fibers replaced the original framework. Generalized lymph node involvement at autopsy showed preservation of the architecture, both in cases with total replacement of the lymphoid population by abnormal cells and in cases with involvement by single cells or small groups of cells. Partial involvement occurred chiefly in the medullary zone, probably the result of hematogenous dissemination from the vascular plexus in medullary cords. Further abnormal cell development was linked to trabecular infiltration. The extensive involvement seen at autopsy took place by lymphatic dissemination. In lymph nodes studied at autopsy an abnormal immunoblastic reaction was observed. At first these abnormal cells were suspected of representing the myeloproliferative malignant disease, but the application of special staining techniques revealed their polyclonal cytoplasmic immunoglobulins. The chloroacetate esterase stain and the immunohistochemical stains for muramidase and hemoglobulin were especially useful in demonstrating that myeloproliferative diseases develop in the environment found in lymph nodes.

Adult↗
PubMed2000

Soft-tissue vascular anomalies: utility of US for diagnosis.

PURPOSE: To determine the ultrasonographic (US) features that distinguish soft-tissue hemangioma from vascular malformation and one type of malformation from another. MATERIALS AND METHODS: Eighty-seven vascular anomalies were evaluated by means of US. Lesions were assessed for the presence of solid tissue and abnormal arteries, veins, or cysts. Vessel density, peak flow velocities, and resistive indexes were compared. RESULTS: There were 49 hemangiomas and 38 vascular malformations. A significantly greater proportion of hemangiomas (48 of 49) compared with vascular malformations (zero of 38) consisted of a solid-tissue mass (P < .001). Vessel density was comparable for hemangioma and arteriovenous malformation (AVM) but significantly greater compared with the other vascular malformations (P < .001 in each case). No differences in mean arterial peak velocity were detected between hemangiomas and malformations. Mean venous peak velocity was significantly higher for AVM than for other vascular malformations and hemangioma. Mean resistive index was greater for lymphatic malformation than for hemangioma or AVM. Abnormal veins, arteries and veins, or cysts were univariate predictors for distinguishing between venous, arteriovenous, and lymphatic malformations (P < .001 in all cases). Solid-tissue mass was the only multivariate predictor for differentiating hemangioma from vascular malformation (likelihood ratio test = 109.8, P < .001). CONCLUSION: US can be used to distinguish hemangioma from vascular malformation and detect arterial flow. These distinctions are critical for subsequent management and assessing prognosis.

Adolescent↗