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Lymphangioleiomyomatosis (LAM) is a rare, progressive and systemic disease that typically results in cystic lung destruction. It predominantly affects women, especially during childbearing years. [ 1 ]
There is no standard approach to the treatment of lymphangiomatosis and treatment often is aimed at reducing symptoms. [2] [17] Surgical intervention may be indicated when complications arise and a number of reports of response to surgical interventions, medications, and dietary approaches can be found in the medical literature. [2] [16] [17]
Sirolimus, also known as rapamycin and sold under the brand name Rapamune among others, is a macrolide compound that is used to coat coronary stents, prevent organ transplant rejection, treat a rare lung disease called lymphangioleiomyomatosis, and treat perivascular epithelioid cell tumour (PEComa).
Treatment for cystic hygroma involves the removal of the abnormal tissue; however, complete removal may be impossible without removing other normal areas. Surgical removal of the tumor is the typical treatment provided, with the understanding that additional removal procedures will most likely be required as the lymphangioma grows.
Most people with benign angiomyolipomas do not show signs or have symptoms. However, symptoms can occur if the dilated blood vessels in an angiomyolipoma rupture; this is called a retroperitoneal hemorrhage. This can cause pain in the back, nausea and vomiting. Some long-term effects are anemia, hypertension, and chronic kidney disease.
Lymphohemangioma is a disease characterized by swelling of the lymph nodes and blood vessels.It is variously described as a "mixture of clear fluid and blood-filled cysts", [citation needed] a mass of abnormal swollen veins and lymph nodes, or a tumorous growth of lymph and blood vessels.
Leiomyomas do not typically require treatment unless they cause pain. [6] The skin lesions may be difficult to treat as they tend to recur after excision or destructive treatment. Drugs which affect smooth muscle contraction, such as doxazosin, nitroglycerine, nifedipine and phenoxybenzamine, may provide pain relief.
Signs and symptoms include fever, chills, shivers, feeling sick, headache, fast heart beat, low blood pressure, breathing fast, flushing of skin, muscle aches, and worsening of skin lesions. [1] It may sometimes be mistaken as an allergy to the antibiotic. [1]