Developmental Disorders of the Lymphatics

An information blog for disorders of the lymphatics. For all articles, please click on "Archives" - Due to spammers, I will no longer allow comments, sorry.

Wednesday, April 10, 2013

I have not been well as soon as I am I will again be posting 04/10/13


I have not been well as soon as I am I will again be posting 04/10/13

Sunday, March 03, 2013

Is lymphatic status related to regression of inflammation in Crohn's disease?


Is lymphatic status related to regression of inflammation in Crohn's disease?


2012

Source

Francesco Tonelli, Francesco Giudici, Gadiel Liscia, Department of Clinical Physiopathology, University of Florence, Surgical Unit, 50134 Florence, Italy.

Abstract


AIM:

To investigate the status of the lymphatic vessels in the small bowel affected by Crohn's disease (CD) at the moment of surgery.

METHODS:

During the period January 2011-June 2011, 25 consecutive patients affected by CD were operated on in our Institution. During surgery, Patent Blue V was injected subserosally and the way it spread along the subserosa of the intestinal wall, through the mesenterial layers towards the main lymphatic collectors and eventually to the lymph nodes was observed and recorded. Since some patients had been undergone strictureplasty at previous surgery, we also examined the status of intestinal lymph vessels after previous strictureplasties. The same procedure was performed in a control group of 5 patients affected by colorectal cancer. Length of lesions, caliber, maximal thickness of the diseased intestinal wall, thickness of the wall at injection site and thickness of the mesentery were evaluated at surgery.

RESULTS:

We observed three features after the injection of Patent Blue V in the intestinal loops: (1) Macroscopically healthy terminal ileum of patients with CD or colon cancer showed thin lymphatic vessels linearly directed toward the mesentery; (2) In mild lesions in which the intestinal wall did not reach 8 mm of thickness, we observed short, wide and tortuous lymphatic vessels directed longitudinally along the intestinal axis toward disease-free areas and then transversally toward the mesentery; and (3) Injection in the severely affected lesions, that had a thickness of the intestinal wall over 10 mm, did not show any feature of lymphatic vessels at least on the subserosal surface. There was a correlation between the thickness of the parietal wall and the severity of the lymphatic alterations. Normal lymphatic vessels were observed at previous strictureplasties in the presence of complete regression of the inflammation.

CONCLUSION:

Injection of Patent Blue V in the intestinal wall could help distinguish healthy tracts of the small bowel from those macroscopically borderline.

KEYWORDS:

Crohn’s disease, Inflammation, Intestinal wall, Lymphatic vessels, Mesentery, Patent blue V, Strictureplasty, Surgery, Thickness

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Sunday, February 17, 2013

Regulation of lymphatic vascular morphogenesis: Implications for pathological (tumor)lymphangiogenesis.


Regulation of lymphatic vascular morphogenesis: Implications for pathological - tumor lymphangiogenesis.


Feb 2013

Source

Lymphatic Development Laboratory, Cancer Research UK London Research Institute, 44 Lincoln's Inn Fields, London WC2A 3LY, UK.

Abstract

Lymphatic vasculature forms the second part of our circulatory system that plays a critical role in tissue fluid homeostasis. Failure of the lymphatic system can lead to excessive accumulation of fluid within the tissue, a condition called lymphedemaLymphatic dysfunction has also been implicated in cancer metastasis as well as pathogenesis of obesity, atherosclerosis and cardiovascular disease. Since the identification of the first lymphatic marker VEGFR-3 and growth factor VEGF-C almost 20 years ago, a great progress has been made in understanding the mechanisms of lymphangiogenesis. This has been achieved largely through characterization of animal models with specific lymphatic defects and identification of genes causative of human hereditary lymphedema syndromes. In this review we will summarize the current understanding of the regulation of lymphatic vascular morphogenesis, focusing on mechanisms that have been implicated in both developmental and pathological (tumor) lymphangiogenesis.

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Friday, February 08, 2013

Incorporating measured valve properties into a numerical model of a lymphatic vessel.


Incorporating measured valve properties into a numerical model of a lymphatic vessel.


Feb 2013

Source

a School of Mathematics and Statistics, University of Sydney, New South , Wales , 2006 , Australia.

Abstract

An existing lumped-parameter model of multiple lymphangions (lymphatic vascular segments) in series is adapted for the incorporation of recent physiological measurements of lymphatic vascular properties. The new data show very marked nonlinearity of the passive pressure-diameter relation during distension, relative to comparable blood vessels, and complex valve behaviour. Since lymph is transported as a result of either the active contraction or the passive squeezing of vascular segments situated between two one-way valves, the performance of these valves is of primary importance. The valves display hysteresis (the opening and closing pressure drop thresholds differ), a bias to staying open (both state changes occur when the trans-valve pressure drop is adverse) and pressure-drop threshold dependence on transmural pressure. These properties, in combination with the strong nonlinearity that valve operation represents, have in turn caused intriguing numerical problems in the model, and we describe numerical stratagems by which we have overcome the problems. The principal problem is also generalised into a relatively simple mathematical example, for which solution detail is provided using two different solvers.

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Friday, February 01, 2013

Noonan syndrome.


Noonan syndrome.


Jan 2013

Source

Department of Cardiology and Division of Genetics, Children's Hospital Boston, Boston, MA 02115, USA. amy.roberts@cardio.chboston.org

Abstract


Noonan syndrome is a genetic multisystem disorder characterised by distinctive facial features, developmental delay, learning difficulties, short stature, congenital heart disease, renal anomalies, lymphatic malformations, and bleeding difficulties. Mutations that cause Noonan syndrome alter genes encoding proteins with roles in the RAS-MAPK pathway, leading to pathway dysregulation. Management guidelines have been developed. Several clinically relevant genotype-phenotype correlations aid risk assessment and patient management. Increased understanding of the pathophysiology of the disease could help development of pharmacogenetic treatments.


See also:

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A pathway for unicellular tube extension depending on the lymphatic vessel determinant Prox1 and on osmoregulation.


A pathway for unicellular tube extension depending on the lymphatic vessel determinant Prox1 and on osmoregulation.


Jan 2013

Source

1] IGBMC, Development and Stem Cells Program, CNRS (UMR7104)/INSERM (U964)/Université de Strasbourg, 1 rue Laurent Fries, BP.10142, 67400 Illkirch, France [2].

Abstract


The mechanisms regulating the extension of small unicellular tubes remain poorly defined. Here we identify several steps in Caenorhabditis elegans excretory canal growth, and propose a model for lumen extension. Our results suggest that the basal and apical excretory membranes grow sequentially: the former extends first like an axon growth cone; the latter extends next as a result of an osmoregulatory activity triggering peri-apical vesicles (a membrane reservoir) to fuse with the lumen. An apical cytoskeletal web including intermediate filaments and actin crosslinking proteins ensures straight regular lumengrowth. Expression of several genes encoding proteins mediating excretory lumen extension, such as the osmoregulatory STE20-like kinase GCK-3 and the intermediate filament IFB-1, is regulated by ceh-26 (here referred to as pros-1), which we found essential for excretory canal formation. Interestingly, PROS-1 is homologous to vertebrate Prox1, a transcription factor controlling lymphatic vessel growth. Our findings have potential evolutionary implications for the origin of fluid-collecting organs, and provide a reference for lymphangiogenesis.

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Adiponectin receptor expression in gastric carcinoma: implications in tumor development and progression.


Adiponectin receptor expression in gastric carcinoma: implications in tumor development and progression.


Jan 2013

Source

Department of Pathology, Seoul National University Bundang Hospital, 173-82 Gumiro, Bundang-gu, Seongnam, Gyeonggi, 463-707, South Korea.

Abstract


PURPOSE:

Adiponectin, an adipocyte-secreted endogenous insulin sensitizer, appears to play an important role in progression of several malignancies. Expression of adiponectin receptors-AdipoR1 and AdipoR2-has been documented in gastric cancer (GC) cell lines, but its role in GCs is still controversial. We investigated expression level of 2 adiponectin receptors and correlated their expression with prognosis in GC patients.

METHODS:

We immunohistochemically evaluated AdipoR1 and AdipoR2 expression in 59 non-neoplastic gastric mucosas, 48 gastric adenomas, 250 GCs, and 58 lymph nodes involved by metastatic GC and assessed its association with clinicopathologic characteristics.

RESULTS:

Expression rates of both receptors increased stepwise in non-neoplastic gastric mucosa, gastric adenoma, intestinal-type GC, and metastatic GC (p < 0.001). AdipoR1 and AdipoR2 expression was observed in 85 (34.0 %) and 118 (47.2 %) GC cases, respectively. Expression rates were higher in intestinal-type GC than in diffuse-type GC (p < 0.001 and 0.016, respectively). AdipoR1 and AdipoR2 expression was more frequent in advanced GC than in early GC (p < 0.001, each) and was associated with lymphatic invasion (p = 0.046 and 0.001, respectively). AdipoR2 expression was associated with poor overall and disease-free survival (p = 0.001 and 0.007, respectively). AdipoR1 expression was associated with poor disease-free survival for intestinal-type GC patients (p = 0.046). In multivariate analysis, AdipoR2 was an independent prognostic factor for intestinal-type GC (p = 0.017).

CONCLUSIONS:

Adiponectin receptor expression is related to GC development and progression, especially intestinal-type GC. Thus, adiponectin receptor expression can serve as a prognostic marker in GC patients.

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Monday, January 28, 2013

Nonspecific granulomatous inflammation in Crohn's disease.


Nonspecific granulomatous inflammation in Crohn's disease.


2012

[Article in Russian]
[No authors listed]

Abstract


A comparative morphological study of intestinal wall tissues in such chronic colonic diseases, such as Crohn's disease, ulcerative colitis, and catarrhal rectal fistulas, allows the formation of giant cells of foreign bodies and their granulomas and sarcoid-type ones to be nonspecific. Their spread through and outside the colon is due to the migration of foreign bodies along the lymphatic vessels. Foreign inclusions of different shapes and structures in the cytoplasm of giant cells suggest that the colon contains the multiple particles of varying antigenic nature, which induce a unified morphological response medicated by innate and adaptive immunity cells. Consequently, the universally accepted substantiation of the diagnosis of Crohn's disease by the presence of granulomas is unconvincing.

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Saturday, January 19, 2013

Hemangioendothelioma.


Hemangioendothelioma.


Feb 2013

Source

Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain. Electronic address: lrequena@fjd.es.

Abstract


Hemangioendothelioma is the term used to name those vascular neoplasms that show a borderline biological behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas. Although originally spindle cell hemangioendothelioma was proposed as a specific clinicopathologic variant of hemangioendothelioma, currently, it is considered as an entirely benign lesion, and thus, the name spindle cell hemangioma seems to be the most accurate for this lesion. Authentic hemangioendotheliomas involving the skin and soft tissues include papillary intralymphatic angioendothelioma (also known as Dabska tumor), retiform hemangioendothelioma, kaposiform hemangioendothelioma, epithelioid hemangioendothelioma, pseudomyogenic hemangioendothelioma (also known as epithelioid sarcoma-like hemangioendothelioma), and composite hemangioendothelioma. Each of these neoplasms exhibit characteristic histopathologic features. The most characteristic finding of papillary intralymphatic hemangioendothelioma consists of papillary tufts, with a central hyaline core lined by hobnail-like endothelial cells protruding into the lumina. Retiform hemangioendothelioma is an infiltrative neoplasm composed of elongated arborizing vessels, arranged in an anastomosing pattern that resembles that of the rete testis, and lined by a single layer of hobnail-like endothelial cells that protrude within the narrow lumina. Kaposiform hemangioendothelioma is composed of several solid poorly circumscribed nodules, and each nodule is composed of a mixture of small capillaries and solid lobules of endothelial cells arranged in a glomeruloid pattern. A frequent finding consists of the presence of areas of lymphangiomatosis adjacent to the solid nodules. Epithelioid hemangioendothelioma is composed of cords, strands, and solid aggregates of round, oval, and polygonal cells, with abundant pale eosinophilic cytoplasm, vesicular nuclei, and inconspicuous nucleoli, embedded in a fibromyxoid or sclerotic stroma. Many neoplastic cells exhibit prominent cytoplasmic vacuolization as an expression of primitive vascular differentiation. Pseudomyogenic hemangioendothelioma is a poorly circumscribed, fascicular lesion with infiltrative borders composed of round or oval neoplastic cells, with vesicular nuclei and inconspicuous nucleoli, and ample homogeneous eosinophilic cytoplasm, giving them a rhabdomyoblastic appearance. Finally, composite hemangioendothelioma is the term used to name locally aggressive vascular neoplasms of low-grade malignancy showing varying combinations of benign, low-grade malignant, and high-grade malignant vascular components. From the immunohistochemical point of view, proliferating cells of all hemangioendotheliomas express a lymphatic endothelial cell immunophenotype. Most hemangioendotheliomas are low-grade vascular neoplasms, with a tendency to recur locally and a low metastatic potential, mostly to regional lymph nodes. Epithelioid hemangioendothelioma, especially large lesions and those located in deep soft tissues, seems to have a more aggressive biological behavior.

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