Cytomegalovirus-associated protein-losing enteropathy resulting from lymphangiectasia in an immunocompetent child.

Cytomegalovirus-associated protein-losing enteropathy resulting from

lymphangiectasia in an immunocompetent child.

Jpn J Infect Dis. 2009 May

Hoshina T, Kusuhara K, Saito M, Hara T, Mtsuura S, Yano T, Aoki T, Hara T.

Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan. hoshina@pediatr.med.kyushu-u.ac.jp

An immunocompetent 8-year-old boy with cytomegalovirus (CMV)-associated transient protein-losing enteropathy (PLE) is described. Colonoscopic examination revealed lymphoid hyperplasia of the terminal ileum. Histological examination of the biopsied specimens showed marked dilation of the lymphatic vessels. Primary CMV infection was demonstrated by serological test and polymerase chain reaction. The child had complete resolution of the disease without antiviral treatment. The present case suggests the etiologic role of CMV infection in PLE resulting from intestinal lymphangiectasia in childhood.

NIH

P13K 1-phosphatidylinositol 3-kinase definition and studies

P13K 1-phosphatidylinositol 3-kinase definition

<enzyme>

<cell biology> An enzyme that catalyses the conversion of phosphatidylinositol to phosphatidylinositol 3-phosphate. This is the first committed step in the biosynthesis of phosphatidylinositol 3,4-bisphosphate and phosphatidylinositol 3,4,5-trisphosphate. This pathway is thought to play a critical role in DNA repair, v(d)j recombination and cell cyle checkpoints.37.

Chemical name: ATP:1-phosphatidyl-1D-myo-inositol 3-phosphotransferase

Registry number: EC 2.7.1.137

Online Medical Dictionary

Additional studies on its role:

Effects of Tyroserleutide on Gene Expression of Calmodulin and P13K in Hepatocellular Carcinoma

Oncogenic P13K Deregulates Transcription and Translation

A role for phosphoinositol-3-kinase (P13K) in the stimulation of hepatic taurocholate uptake by cyclic AMP

Laminin-332 promotes the invasion of oesophageal squamous cell carcinoma via P13K activation

Patent application title: THIAZOLOPYRIMIDINE P13K INHIBITOR COMPOUNDS AND METHODS OF USE

Expression Status and Mutational Analysis of the PTEN and P13K Subunit Genes in Ovarian Granulosa Cell Tumors

High D-glucose alters PI3K and Akt signaling and leads to endothelial cell migration,

proliferation and angiogenesis dysfunction

Organ-specific lymphangiectasia, arrested lymphatic sprouting, and maturation defects P13K gene

Organ-specific lymphangiectasia, arrested lymphatic sprouting, and maturation defects resulting from gene-targeting of the PI3K regulatory isoforms p85, p55, and p50

Dev Dyn. 2009 Oct

Mouta-Bellum C, Kirov A, Miceli-Libby L, Mancini ML, Petrova TV, Liaw L, Prudovsky I,Thorpe PE, Miura N, Cantley LC, Alitalo K, Fruman DA, Vary CP.

Center for Molecular Medicine, Maine Medical Center Research Institute, Scarborough, Maine, USA.

email: Calvin P.H. Vary (varyc@mmc.org)

The phosphoinositide 3-kinase (PI3K) family has multiple vascular functions, but the specific regulatory isoform supporting lymphangiogenesis remains unidentified. Here, we report that deletion of the Pik3r1 gene, encoding the regulatory subunits p85alpha, p55alpha, and p50alpha impairs lymphatic sprouting and maturation, and causes abnormal lymphatic morphology, without major impact on blood vessels. Pik3r1 deletion had the most severe consequences among gut and diaphragm lymphatics, which share the retroperitoneal anlage, initially suggesting that the Pik3r1 role in this vasculature is anlage-dependent. However, whereas lymphatic sprouting toward the diaphragm was arrested, lymphatics invaded the gut, where remodeling and valve formation were impaired. Thus, cell-origin fails to explain the phenotype. Only the gut showed lymphangiectasia, lymphatic up-regulation of the transforming growth factor-beta co-receptor endoglin, and reduced levels of mature vascular endothelial growth factor-C protein. Our data suggest that Pik3r1 isoforms are required for distinct steps of embryonic lymphangiogenesis in different organ microenvironments, whereas they are largely dispensable for hemangiogenesis.

Wiley InterScience

Reversal of protein-losing enteropathy after heart transplantation in young patients.

Reversal of protein-losing enteropathy after heart transplantation in young patients

Rev Esp Cardiol. 2009 August

Soriano JR, Grima EZ, Vives MA, Sáez AO, Dolz LM, Bonet LA, Pérez MA, Sanz AS.

Servicio de Cardiología, Hospital Universitario La Fe, Valencia, Spain. ximo@ipeuropa.com

Protein-losing enteropathy is a rare but life-threatening complication that occurs in some patients who develop intestinal lymphangiectasis secondary to increased systemic venous pressure. Although different forms of treatment have been tried, with varying results, the majority were reported to be unsuccessful. The aim of this study was to demonstrate that heart transplantation may be an appropriate therapeutic option for patients who do not respond to medical treatment. At our center, we performed heart transplantations in three patients with this condition. The mean follow-up period was 11+/-2 months. No patient died and the enteropathy regressed in all three.

Cardiologia

Lighthouse Lymphedema Network

I wanted to make a special announcement that the Lighthouse Lymphedema Network
is now on Facebook.

You can join by going to: Lighthouse Facebook

There is also an area where you can post and participate.

AND - don't forget our upcoming program in October.

see the details for that at:

Lighthouse Lymphedema Network

**yes, I'm on it too :-)**

Pat

Clinical significance of duodenal lymphangiectasia incidentally found during routine upper gastrointestinal endoscopy.

Clinical significance of duodenal lymphangiectasia incidentally found during routine upper gastrointestinal endoscopy.

Endoscopy. 2009 Jun

Kim JH, Bak YT, Kim JS, Seol SY, Shin BK, Kim HK.
Department of Gastroenterology, Korea University Guro Hospital, Seoul, Korea.

BACKGROUND AND STUDY AIM: Although duodenal lymphangiectasia in individuals without clinical evidence of malabsorption has been reported, the prevalence and clinical significance in this situation are not yet known. The aim of this study was to evaluate the prevalence and clinical significance of incidentally found duodenal lymphangiectasia.

PATIENTS AND METHODS: A retrospective review of medical records was undertaken for consecutive patients who had undergone diagnostic upper endoscopy between January 2005 and June 2006. A prospective study was then performed in consecutive individuals undergoing routine upper endoscopy for health examination between July 2006 to October 2006.

Endoscopic features of duodenal lymphangiectasia were classified into three types: (1) multiple scattered pinpoint white spots; (2) diffuse prominent villi with whitish-discolored tips; and (3) focal small whitish macule or nodule. The histologic grade of duodenal lymphangiectasia was classified according to the depth and severity of lymphatic duct dilatations. Prevalence and clinical data of incidentally found duodenal lymphangiectasia were evaluated in the retrospective and prospective studies.

RESULTS: Among 1866 retrospective cases, duodenal lymphangiectasia was endoscopically suspected in 59 cases (3.2%), and histologically confirmed in 35 cases (1.9%). No clinical evidence of malabsorption was noted in the duodenal lymphangiectasia cases. The "scattered pinpoint white spots" type was the most frequently found endoscopic feature (40.0%). Duodenal lymphangiectasia was persistent in seven of 10 individuals who underwent repeat endoscopy after a median of 12 months. Among 134 prospective cases, duodenal lymphangiectasia was histologically confirmed in 12 cases (8.9%). There was no significant clinical difference between groups with and without duodenal lymphangiectasia. Lymphatic duct dilatation was histologically more severe in the "focal small whitish macule or nodule" type than in the other types.

CONCLUSION: Duodenal lymphangiectasia without clinical evidence of malabsorption is not extremely rare among cases undergoing routine upper gastrointestinal endoscopy.

eThieme Journal

Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts.

Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts.

Gut. 2000 Aug

Bouhnik Y, Etienney I, Nemeth J, Thevenot T, Lavergne-Slove A, Matuchansky C.
Department of Gastroenterology and Hepatology, Hôpital Lariboisière, Paris, France. yoram.bouhnik@lrb.ap-hop-paris.fr

As only a handful of lymphoma cases have been reported in conjunction with primary intestinal lymphangiectasia, it is not yet clear if this association is merely fortuitous or related to primary intestinal lymphangiectasia induced immune deficiency. We report on two female patients, 50 and 58 years old, who developed small intestinal high grade B cell lymphoma a long time (45 and 40 years, respectively) after the initial clinical manifestations of primary intestinal lymphangiectasia. They presented with a longstanding history of fluctuating protein losing enteropathy, multiple cutaneous plane warts, and markedly dilated mucosal and submucosal lymphatic channels in duodenal biopsies. One had a large ulcerated tumour of the proximal ileum and the other diffuse ileal infiltration. In both, histological examination showed centroblastic high grade B cell lymphoma associated with duodenojejuno-ileal mucosal and submucosal lymphangiectasia. They were subsequently successfully treated with surgery and postoperative chemotherapy (AVmCP: adriamycin, cyclophosphamide, Vm26, and prednisolone), and chemotherapy alone (PACOB: adriamycin, cyclophosphamide, vincristine, bleomycine, and prednisolone), respectively. A three year follow up in both cases showed persistent diffuse lymphangiectasia without evidence of lymphoma. The present findings support the hypothesis that primary intestinal lymphangiectasia is associated with lymphoma development.

Gut Online

Protein-losing enteropathy in a case of nodal follicular lymphoma without a gastrointestinal mucosal lesion.

Protein-losing enteropathy in a case of nodal follicular lymphoma without a gastrointestinal mucosal lesion.
Intern Med. 2008
Kaneko H, Yamashita M, Ohshiro M, Ohkawara Y, Matsumoto Y, Nomura K, Horiike S, Yokota S, Taniwaki M.
Department of Hematology, Aiseikai-Yamashina Hospital, Kyoto. hp@aiseikaihp.or.jp

Protein-losing enteropathy (PLE) is characterized by gastrointestinal loss of serum protein. It is usually caused by hypersecretion from a tumor, ulcer, or long standing lymphangiectasia. However, we report a 47-year-old man of peritoneal nodal follicular lymphoma who developed PLE with none of them. Oozing of whitish fluid from duodenal bulbar mucosa was endoscopically seen, resulting in continuous loss of protein. Chemotherapy was effective and PLE was rapidly diminished. Nodal lymphoma lesion was considered to disturb lymphatic flow and to regurgitate it to duodenal mucosa. To our knowledge, this is the second report of a lymphoma patient presenting PLE without a gastrointestinal mucosal lesion.

Internal Medicine

Georgia Lymphedema Education and Awareness Program

11th Annual Lymphedema Education & Awareness Program

An educational and awareness conference for patients, caregivers and professionals!

Where?

Piedmont Hospital

Piedmont Hospital

Richard H. Rich Auditorium

1968 Peachtree Road, NW, Building 77

Atlanta, Georgia



When?

Saturday, October 18, 20087:30 am - 5:30 pm

Program includes

What to expect of tissue after radiation?

What is the physiology response of radiation?

What does radiation do to the lymph nodes? - Peter Rossi, MD

How does vascular flow affect the lymphatics? - Ken Harper, MD

Expectations of surgery. - Christopher Hart, MD, FACSThe Lymphatic System, Wound Care,

Infections and Treatment - Paula Stewart, MDParent Networking

Aquatic Exercises

The Connection of obesity and increased swelling in people with lymphedema and lipedema.and more.

Additional information and registration form - Home website - Lighthouse Lymphedema Network

See you there - Pat

Lympangiectasia of the vulva accompanying congenital lymphedema

Lympangiectasia of the vulva accompanying congenital lymphedema
April 2009

M Ihsan Okur1, Rüştü Köse2, A Mustafa Yıldırım1, Bengü Çobanoğlu3

Dermatology Online Journal 15 (4): 13 1. Assistant Professor, Department of Plastic and Reconstructive Surgery, Fırat Medical Center, Firat (Euphrates) University, Elazig, Turkey2. Assistant Professor, Department of Plastic and Reconstructive Surgery, Harran University Medical Center, Şanlıurfa, Turkey. rkose@harran.edu.tr3.
Assistant Professor, Department of Pathology

Abstract

Lymphangiectasia is a benign condition with multiple dilated lymph vessels in the dermis. Vulvar localization is rare and mostly depends on disturbing the lymphatic flow. We present a patient with vulvar lesions and ipsilateral congenital lower extremity lymphedema. Surgical excision eliminated the lymphangiectasia and improved the appearance of the edematous vulva.

Introduction

Vulvar lymphangiectasia is not usually seen as a result of congenital lymphedema. Lymphangioma circumscriptum, a subtype of lymphangioma, is mainly a congenital malformation that presents in infancy but may be seen at any age. An acquired lesion, especially one related to the obstruction of lymphatic circulation, is defined as lymphangiectasis; however it is histologically indistinguishable from lymphangioma circumscriptum.
Lymphangiectasia of the vulva is a rare disease and usually reported following surgery and radiotherapy, performed because of a genital malignant tumor [1]. Long-standing Crohn disease with fistulae and tuberculosis lymphadenitis are the other associated conditions [1, 2, 3, 4]. Primary lymphedema is an unusual cause of vulvar lymphangiectasia. We present a case of vulvar lymphangiectasia with ipsilateral congenital lower extremity lymphedema.

Case Report

A 27-year-old woman was referred for treatment of a skin lesion on her right labium majus. The plaque had appeared initially at the age of 19. Sometimes vesicles arose on the surface from which clear and occasionally bloody fluid oozed. She underwent a few bouts of vulvar cellulitis. Four years prior, she had been seen in another dermatology clinic and was given the clinical diagnosis of "infected lymphangioma of the vulva," which was treated with antibiotics. Congenital lympedema along the entire right leg was noted. The lymphedema (Fig. 1) was apparent soon after birth and she regularly used a pressure garment. Genital examination revealed a red and skin-colored plaque with shining, clustered papules on the right labium majus and ipsilateral vulvar edema (Fig. 2). This edema deformed the appearance of the vulva, which was the main complaint of the patient. The growth was excised, including the edematous subcutaneous tissue, under general anesthesia. A negative pressure drain was placed and tissues were sutured.

The histopathological examination of the excised tissue showed many thin-wall lumenal structures with irregular size and shape, lined by a monolayer of endothelium in the upper dermis (Fig. 3). Postoperative recovery was uneventful and the surgical wound healed well.

After the surgery, the symmetry of the vulva was improved. Although a thin scar remained along the labium majus, the aesthetic outcome was good (Fig. 4) and the patient was satisfied. The patient was followed up for 31 months. Recurrence and infection were not observed during this period.

Comment

Vulvar lymphangiectasia is mainly associated with obstruction of the lymphatic flow. The lesions typically arise approximately 7-15 years after lymph node dissection and radiotherapy of the genitalia [1, 5, 6, 7, 8]. De novo acquired lesions without lymphatic disturbance are less frequently seen. In some cases, lymphangiectasia appears following lymphedema of the vulva or lower extremity [3, 4, 5, 6, 9]. However, the condition may arise without adjacent lymphedema [3].

Although deep lymphangiomas are seen in primary lymphedema cases, reports detailing subsequent lymphangiectasia of vulva are very few [2, 10]. Buckley and Barnes report a 35-year-old patient who had lymphedema since the age of 14 and underwent two cellulitis attacks. Thereafter, lymphangiectasia on the vulva appeared in the same year [10]. Another patient with late onset lymphedema in the right leg also presented with lymphangiectasia on the labium majus [2]. The lesions emerged three years after the lymphedema.

The mechanism behind development of the vulvar lymphangiectasia in our patient accompanied by congenital lymphedema is probably similar. A 13-year-old boy has been described who presented exhibited scrotal lymphangiectasia with congenital contralateral leg lymphedema [11].

He also experienced recurrent scrotal infections after the lymphangiectasia appeared. Our patient had congenital lymphedema and suffered episodes of cellulitis before and after the vulvar lymphangiectasia appeared.

Lymphangiectasia may be actually expected in primary lymphedema patients because lymphatic obstruction is an important etiologic factor in its etiology. The cause of the higher rate in acquired lymphatic damage may be because of the rapidly increasing pressure when there is a sudden blockage of the superficial lymphatic system. In addition, recurrent cellulitis further damages lymphatic vessels, increases lymphatic pressure, and induces formation of lymphangiectasia in lymphedema patients.

Lymphangiectasia on the vulva can be easily misdiagnosed, especially as viral warts [1-5, 7, 8, 12]. Oozing vesicules or papules and recurrent infections should bring into mind the possibility of lymphangiectasia.

Excisional surgery and carbon dioxide laser are two major treatment modalities in addition to cryotherapy, electrocoagulation, and sclerosing agent injection [13]. Whereas carbon dioxide laser only vaporizes the lesion superficially, it may seal the underlying lymph vessels and diminish recurrence [9, 14]. The laser therapy can be repeated if any recurrences occur. Delayed wound healing, scars, and even keloid formation may be seen after laser therapy [1, 14]. Excisional surgery eliminates the abnormal subcutaneous lymph vessels and cisterns, and corrects of the aesthetic appearance of the edematous vulva. It is the only plausible choice in the therapy of advanced disease [13].

DermatologyOnline

Lymphangiectasias of the skin: victims of confusing nomenclature

Lymphangiectasias of the skin: victims of confusing nomenclature
Clin Exp Dermatol. 2009 May
Verma SB.
Nirvana Skin Clinic, Vadodara, Gujarat, India.

Summary Lymphangiectasias are known by a variety of names, in the dermatology literature, including lymphangiectasis, acquired lymphangiomas, secondary lymphangiomas and acquired lymphangioma circumscriptum, which has led to confusion. The condition itself, especially in the genital region, is difficult to diagnose. This article attempts to resolve the issues of the confusing nomenclature and reviews the condition, which can arise under a variety of clinical circumstances. The relevant anatomical and histological details are described, with relevant clinical illustrations, to facilitate understanding of the aetiopathogenesis of this enigmatic condition. The available medical and non-medical treatments are discussed.

WileyInterScience