Primary Intestinal Lymphangiectasia: Four Case Reports and a Review of the Literature

Wen, Jie; Tang, Qingya; Wu, Jiang; Wang, Ying; Cai, Wei

doi:10.1007/s10620-010-1161-1

Cited by 75 publications

(118 citation statements)

References 65 publications

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“…The precise mechanism by which these types of IL develop remains unclear although vascular endothelial growth factor receptors 3, C, and D were reported to associate with IL [20]. Even if patients were initially diagnosed as primary IL, approximately 5% were reported to have a malignant transformation of lymphoma, and the average time from primary IL onset to lymphoma diagnosis was 31 years (range 19-45) [21]. Therefore, the latent development of malignant lymphoma should be kept in mind during the follow-up of patients with primary IL, even though initial corticosteroids had been effective.…”

Section: Discussionmentioning

confidence: 99%

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Ohmiya

Nakamura²,

Yamamura³

et al. 2014

Digestion

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Background/Aims: We classified intestinal lymphangiectasia (IL) into two categories, the white and non-white villi types, and evaluated their clinical characteristics and therapeutic responses. Methods: Of the 988 patients who underwent double-balloon enteroscopy, 14 consecutive patients (7 men and 7 women, median age at onset 34 years) were enrolled with immunohistochemically confirmed IL with protein-losing enteropathy. Results: Enteroscopically the white villi type (n = 8) showed white plaques and white-tipped villi were scattered in the small bowel, while non-white villi type (n = 6) showed that apparently normal but under more detailed observation, low and round villi with a normal color were diffused. The serum albumin levels and fecal E₁-antitrypsin clearance before treatment were significantly worse in the non-white villi type (p = 0.017 and 0.039, respectively), whereas the serum immunoglobulin A and M levels were significantly lower in the white villi type (p = 0.010 and 0.046, respectively). At gastroscopy, a non-cirrhotic snakeskin appearance was significantly observed in the non-white villi type (p = 0.015). The corticosteroid response was better in the non-white villi type (p = 0.015). Conclusion: Two distinct subgroups were found in IL. This classification was useful in pathophysiological clustering and in predicting the therapeutic response. i 2014 S. Karger AG, Basel

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Section: Discussionmentioning

confidence: 99%

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Ohmiya

Nakamura²,

Yamamura³

et al. 2014

Digestion

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“…MCT are directly absorbed into the portal venous system, which prevents lacteal engorgement [24] . Dietary intervention is more effective in children than in adults, therefore early diagnosis of intestinal lymphangiectasia and consistent attention to diet are very important for early nutrition and growth [25] . Small bowel resection may be helpful to treat localized disease [26] .…”

Section: Discussionmentioning

confidence: 99%

Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy

Oh¹

2011

WJGE

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Author contributions: Oh TG designed the case report as a main author; Chung JW and Kim HM contributed to provide background knowledge; Han SJ provided many informations about the patient; Lee JS provided genetic information and accessment; Park JY participated in endoscopic evaluation and accessment; Song SY supervised and checked up the manuscript finally as a corresponding author; all authors reviewed and approved the final manuscript. AbstractPrimary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lymphatics and the development of protein-losing enteropathy. Patients with PIL develop hypoalbuminemia, hypocalcemia, lymphopenia and hypogammaglobulinemia, and present with bilateral lower limb edema, fatigue, abdominal pain and diarrhea. Endoscopy reveals diffusely elongated, circumferential and polypoid mucosae covered with whitish enlarged villi, all of which indicate intestinal lymphangiectasia. Diagnosis is confirmed by characteristic tissue pathology, which includes dilated intestinal lymphatics with diffusely swollen mucosa and enlarged villi. The prevalence of PIL has increased since the introduction of capsule endoscopy. The etiology and prevalence of PIL remain unknown. Some studies have reported that several genes and regulatory molecules for lymphangiogenesis are related to PIL. We report the case of a patient with PIL involving the entire small bowel that was confirmed by capsule endoscopy and double-balloon enteroscopy-guided tissue pathology who carried a deletion on chromosome 4q25. The relationship between this deletion on chromosome 4 and PIL remains to be investigated.

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“…Regarding the main complications, it is not clear if malignancy, especially lymphoma, is fortuitous or related to the disease. Among 84 PIL patients reviewed in 2010, four (5%) had malignant lymphoma, and the average time from PIL onset to lymphoma diagnosis was 31 years (range 19-45 years) (7). Lymphoma mainly affects the gastrointestinal tract but may also be seen in the retroperitoneum and the mediastinum.…”

Section: Discussionmentioning

confidence: 99%

Waldmanns disease: a rare cause of protein losing enteropathy in an adult patient

Martins¹,

Gagnaire²,

Rostain³

et al. 2017

Rev Esp Enferm Dig

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Primary intestinal lymphangiectasia or Waldmann's disease is an uncommon cause of protein losing enteropathy with an unknown etiology and is usually diagnosed during childhood. It is characterized by dilation and leakage of intestinal lymph vessels leading to hypoalbuminemia, hypogammaglobulinemia and lymphopenia. Differential diagnosis should include erosive and nonerosive gastrointestinal disorders, conditions involving mesenteric lymphatic obstruction and cardiovascular disorders that increase central venous pressure. Since there are no accurate serological or radiological available tests, enteroscopy with histopathological examination based on intestinal biopsy specimens is currently the gold standard diagnostic modality of intestinal lymphangiectasia. We report a rare case of a primary intestinal lymphangiectasia in a 60-year-old Caucasian female who presented with asymptomatic hypoalbuminemia and hypogammaglobulinemia. After the diagnosis of a protein losing enteropathy, the patient underwent an enteroscopy and biopsies were taken, whose histological examination confirmed dilated intestinal lymphatics with broadened villi of the small bowel. Secondary causes of intestinal lymphangiectasia were excluded and the diagnosis of Waldmann's disease was recorded. The patient was put on a high-protein and low-fat diet with medium-chain triglyceride supplementation with improvement.

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Primary Intestinal Lymphangiectasia: Four Case Reports and a Review of the Literature

Cited by 75 publications

References 65 publications

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy

Waldmanns disease: a rare cause of protein losing enteropathy in an adult patient

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Primary Intestinal Lymphangiectasia: Four Case Reports and a Review of the Literature

Cited by 75 publications

References 65 publications

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy

Waldmanns disease: a rare cause of protein losing enteropathy in an adult patient

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Waldmanns disease: a rare cause of protein losing enteropathy in an adult patient