Lymphangiomatosis is a rare lymphatic system disease that generally affects patients less than 20 years old, although anybody can contract the ailment. Lymphangiomatosis can manifest as a decongestive system, reproductive system, lung, bone, spleen, or liver disease, that affects various tissues. The most common form of this ailment, Diffuselymphangiomatosis, afflicts the bones in as many as 75 percent of reported cases.
Although the exact causes of Lymphangiomatosis are unknown, abnormalities in lymphatic canals, suggesting neoplastic etiologies and hamartomatous origins, are believed to cause this disease characterized by benign focal proliferations of dilated lymphatic canals, aligned by endothelium conjunctive tissues, forming mediastinal masses in the anterior portion of the mediastynum, which spread to the lymph nodes. Thymoma, Lymphoma, Germ Cell, Teratoma, Pheochromocytoma, and Lung Cancers can be included in these masses.
Symptoms Of Lymphangiomatosis:
Symptoms of Lymphangiomatosis may include pericardial effusions, the abnormal accumulations of fluid in the pericardial cavity, leading to increased pressures, that can affect heart functions. Pleural effusions, or excess fluid build-ups in the space surrounding the lungs that can impair breathing by limiting lung expansions, internal bleeding, and ascite fluid accumulations in the abdominal cavity, are other possible symptoms of Lymphangiomatosis.
Lymphangiomatosis symptoms may also include Cyanosis, occasionally described as Blue Skin or Purple Skin, a severe condition indicating a lack of oxygen in the blood supply, respiratory distresses, pulmonary hypertensions in the blood vessels of the lungs caused by airways that are collapsed by fluid, enlarged lungs, lung tumors, and hemihypertrophy, a syndrome where body parts grow excessively large giving asymmetrical appearances.
Methods Of Diagnosing Lymphangiomatosis:
The methods of diagnosing Lymphangiomatosis do not contain a current standard and are typically based on the exhibited symptoms, radiographic findings, and biopsies that are not always possible. Very little is known about Lymphangiomatosis and treatments with vascular growth inhibitors may slow the process of this fatal disease.
Many non-cancerous lymphatic system tumors may develop in patients with Lymphangiomatosis. Surgical removal of these tumors may be possible, but very difficult, if they have spread. Interferon Alpha medication, chemotherapy, and radiation may be useful in treating the ailment. Thoracentesis, the insertation of a tube into the chest to drain excess fluid, and spinal surgeries to relieve pinched nerves, may alleviate some of the pain associated with Lymphangiomatosis.
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