Lymphangiomatosis

Lymphangiomatosis is a rare condition which is characterized by a diffuse proliferation of lymphatic vessels. This may include internal organs, bone, soft tissue and skin concern. The strongest distribution can be found in children and young adults. Symptoms and chances of recovery will vary greatly between individuals and as a function of the affected organs. Although little is known about the causes, it shall be presumed that it is a rare growth failure that results in an abnormal development of the lymphatic system.

The name Lymphangiomatosis has its origin in the words of lymph - (Description of the lymphatic system ), angio ( vessel) - oma (tumor or cyst ), Tosi ( state). Other names for this or similar diseases are: general lymphangioma, cystic angiomatosis systemic, multiple Lymphangiectasias, general or diffuse lymphatic malformation, general lymphatic anomaly.

A single collection of lymphatic vessels or cysts called lymphangioma. Lymphangiomatosis describes cases where a lymphangioma is not present as a singular mass, but where the problem widespread or present at different locations (eg, when different parts of the body are affected).

The general Lymphangiomatosis was first described in 1828.

Signs and Symptoms

Almost every part of the body can be affected by Lymphangiomatosis. Only the central nervous system is not affected due to lack of lymphatic vessels. Signs and symptoms depend on the body area that is affected.

It cause the following problems: pericardial effusion ( fluid in the heart region ), pleural effusion ( fluid around the lungs cave ), ascites (fluid in the abdominal cavity), fractures, skin lesions, fever and internal bleeding. The effusion is in type chylous ( milky). Symptoms include shortness of breath, cough, trouble breathing, severe pain in the abdomen and pelvic area, and lymphedema (swelling). Although some patients have no symptoms, but show abnormalities in various imaging techniques.

Diagnosis

Currently there is no standard for the diagnosis of this disease. The symptoms, clinical presentation and typical radiographic findings help in the diagnosis. A biopsy is the best possible method, but not in every case. Whole-body MRI provides good ways to determine the spread of the disease. MRI provides better results when compared to CT scans.

Lymphangiomatosis can be confused with other diseases, such as lymphatic displasia, lymphangiectasis and the similar sounding Lymphangioleiomyomatosis (LAM ). LAM occurs mainly in women of childbearing age.

Other diseases associated with Lymphangiomatosis

The Gorham - Stout disease lymphatic vessels grow into the bone, which is usually associated with their resolution. Many patients affected by both diseases, which makes a precise statement. However, there are also cases in which patients are diagnosed with Lymphangiomatosis exclusively or exclusively on the Gorham - Stout disease.

Treatment options

If the abnormal lymphatic tissue can be removed by surgery, then a cure is possible. Because of the infiltrating behavior of the lymphatic vessels in the Lymphangiomatosis However, the problem in most patients can not be solved by surgery. The treatment therefore often focuses on relief of symptoms and tries to stem the progression of the disease. Vascular endothelial growth factors are known to regulate the growth of lymphatic vessels. VEGFR3 protein is important in the formation of the lymphatic system to the member. This means that the treatment with vascular growth inhibitors can mitigate the progression of the disease.

A wide range of additional treatments were applied. These include:

  • Drainage of the area around the lungs and around the heart ( pericardial and pleural drainage)
  • Pleurodesis ( procedure to restrict the space around the lungs to prevent fluid retention)
  • Restricted diet that avoids fat absorption (except medium-chain triglycerides )
  • Drug treatment with: Methylprednisilone, bisphosphonates, alpha interferon, chlorambucil / Etoposide
  • Radiotherapy
  • Amputation
  • Lung transplantation

Forecast

The tumors, which produces this disease are not metastatic like cancer, spreading but still often in the course of time.

The prognosis depends on the affected tissue and spread. If a lot of soft tissue is affected, as well as the presence of a chylothorax ( lymphatic fluid in the lung cavity), then the prognosis is generally relatively poor. As long as only the extremities are affected, the prognosis is usually better.

Research

To date, relatively little is known about this rare disease and there are so far no major research projects in patients with this disease. Case reports represent the primary format of previous publications, namely at Lymphangiomatosis and Gorham 's disease. The number of affected patients is unknown because it is not published for any case reports.

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