Lymphangiomatosis
Lymphangiomatosis (LYMF) is a condition where a lymphangioma is not present in a single localised mass, but in a widespread or multifocal manner.[1] It is a rare neoplasm which results from an abnormal development of the lymphatic system.[2] This may involve one or several organs.
As this disease is often confused with another rare lymphatic disease, lymphangioleiomyomatosis, (LAM), the acronym LYMF was created in January 2005 to 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.
Generalized lymphangiomatosis was first described in 1828.[3]
Contents
Signs and symptoms
Lymphangiomatosis can present with pericardial effusions, pleural effusion, ascites (fluid in the abdominal cavity), and internal bleeding. CT scans on some patients have been similar to lymphoma as tumor-type masses have been found in the mediastinum. 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).
Diagnosis
There is no current standard for diagnosing this condition. The symptoms, clinical presentation, and typical radiographical findings can assist in the diagnosis.[4] A biopsy is the ultimate method for diagnosis, but is not always possible.
LYMF and LAM are easily confused and must be differentiated for an accurate diagnosis. 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.[5]
Common co-morbidities
Lymphangiomatosis is sometimes confused with Gorham's disease (lymph vessels growing into bones), because many patients have both diseases. However, not all patients diagnosed with LYMF have Gorham's and not all patients diagnosed with Gorham's have LYMF.
Treatment
Treatment with vascular growth inhibitors may slow the progress of the disease.
Prognosis
Both LYMF and LAM are known to be fatal eventually, although patients may live for ten years or more.
Research
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 both LYMF and Gorham's Disease. The number of patients is not known, as all patients do not have case reports written about them.
Physicians and scientists researching LAM do not believe that LYMF and LAM are related; however, there has been no formal research to prove this.
References
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fr:Lymphangiomatose- ↑ Hwang, SS; Choi; Park (2009). "Cavernous mesenteric lymphangiomatosis mimicking metastasis in a patient with rectal cancer: a case report". World journal of gastroenterology : WJG. 15 (31): 3947–9. doi:10.3748/wjg.15.3947. PMC 2731261 Freely accessible. PMID 19701979. More than one of
|author2=and|last2=specified (help); More than one of|author3=and|last3=specified (help) - ↑ Marom, EM; Moran, CA; Munden, RF (2004). "Generalized lymphangiomatosis". AJR. American journal of roentgenology. 182 (4): 1068. PMID 15039189.
- ↑ Wong, CS; Chu, TY (2008). "Clinical and radiological features of generalised lymphangiomatosis". Hong Kong medical journal = Xianggang yi xue za zhi / Hong Kong Academy of Medicine. 14 (5): 402–4. PMID 18840914.
- ↑ Agarwal, P.; Matzinger, F.; Seely, J. (2008). "Case 132: Lymphangiomatosis". Radiology. 247 (1): 288–290. doi:10.1148/radiol.2471042092. PMID 18372473.
- ↑ The Lam foundation.org
