Lymphangiomatosis

Lymphangiomatosis (LYMF) is a condition where a lymphangioma is not present in a single localised mass, but in a widespread or multifocal manner. It is a rare neoplasm which results from an abnormal development of the lymphatic system. This may involve one or several organs.

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As this disease is often confused with another rare lymphatic disease (Lymphangioleiomyomatosis or LAM), the acronym LYMF was created in January of 2005 to help physicians as well as the lay person differentiate between the two diseases. The length of the word and difficulty to pronounce provides an easy to remember/pronounce abbreviation in the form of an acronym.

Synonyms

Lymphangiomatosis has been confused with Gorham's disease. Many patients with LYMF also have Gorham's. Labeling the two diseases as synonyms is inaccurate.^{[citation needed]} Not all patients diagnosed with LYMF have Gorham's and not all patients diagnosed with Gorham's have LYMF.

The current information available is provided primarily by case reports.

Physicians and scientists researching LAM do not believe that LYMF and LAM are related - however - there has been no formal research to prove this.

The two diseases (LYMF & LAM) have the potential of being confused. LYMF is frequently diagnosed in infants, children, and adults of both genders, whereas LAM is primarily a disease found in women of child-bearing age.[1]

Both diseases are known to be fatal eventually, although patients may live for ten years or more.

Signs and symptoms

Lymphangiomatosis (LYMF) can present with pericardial, pleural effusion, ascites (fluid in the abdominal cavity), and internal bleeding. Cat scans on some patients have been similar to lymphoma as tumor-type masses have been found in the mediastinum. The masses found are also called lesions as they have are vascular in nature. The fluid involved in these effusions is chylous (milky type fluid) in nature.

LYMF can involve tissues like the lung, spleen, digestive system, and reproductive system. Symptoms include shortness of breath, difficulty breathing in (inhaling), severe pain in the abdominal cavity and pelvic cavity, and lymphedema (swelling).

Some patients diagnosed with Gorham's Disease (Lymph vessels growing into bones known) learn at the same time that they also have LYMF.

Very little is known of these diseases and no formal research has been conducted in patients with this diagnosis. Case reports are the primary format of all publications regarding LYMF and Gorham's Disease. The number of patients is not known as all patients do not have case reports written about them.

Diagnosis

There is no current standard for diagnosing this condition. The symptoms, clinical presentation, and typical radiographical findings can assist in the diagnosis. A biopsy is the ultimate method for diagnosis - but is not always possible.