Lymphangiomatosis
Encyclopedia
Lymphangiomatosis 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.
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.
s, 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).
. A biopsy
is the ultimate method for diagnosis, but is not always possible. The disease can even occur with no painful symptoms at all.
LYMF and LAM are easily confused and must be differentiated for an accurate diagnosis. LYMF is frequently diagnosed in children nearing puberty. Although this disease is not limited to being identified in infants, young children, and adults of both genders, whereas LAM is primarily a disease found in women of child-bearing age.
(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.
Physicians and scientists researching LAM do not believe that LYMF and LAM are related; however, there has been no formal research to prove this.
Lymphangioma
Lymphangiomas are malformations of the lymphatic system, which is the network of vessels responsible for returning to the venous system excess fluid from tissues. These malformations can occur at any age and may involve any part of the body, but 90% occur in children less than 2 years of age and...
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
Lymphatic system
The lymphoid system is the part of the immune system comprising a network of conduits called lymphatic vessels that carry a clear fluid called lymph unidirectionally toward the heart. Lymphoid tissue is found in many organs, particularly the lymph nodes, and in the lymphoid follicles associated...
. This may involve one or several organs.
As this disease is often confused with another rare lymphatic disease, lymphangioleiomyomatosis
Lymphangioleiomyomatosis
Lymphangioleiomyomatosis is a rare lung disease that results in a proliferation of disorderly smooth muscle growth throughout the lungs, in the bronchioles, alveolar septa, perivascular spaces, and lymphatics, resulting in the obstruction of small airways and lymphatics...
, (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.
Signs and symptoms
Lymphangiomatosis can present with pericardial effusionPericardial effusion
Pericardial effusion is an abnormal accumulation of fluid in the pericardial cavity. Because of the limited amount of space in the pericardial cavity, fluid accumulation will lead to an increased intrapericardial pressure and this can negatively affect heart function...
s, pleural effusion
Pleural effusion
Pleural effusion is excess fluid that accumulates between the two pleural layers, the fluid-filled space that surrounds the lungs. Excessive amounts of such fluid can impair breathing by limiting the expansion of the lungs during ventilation.-Pathophysiology:...
, ascites
Ascites
Ascites is a gastroenterological term for an accumulation of fluid in the peritoneal cavity.The medical condition is also known as peritoneal cavity fluid, peritoneal fluid excess, hydroperitoneum or more archaically as abdominal dropsy. Although most commonly due to cirrhosis and severe liver...
(fluid in the abdominal cavity), and internal bleeding. CT scans on some patients have been similar to lymphoma
Lymphoma
Lymphoma is a cancer in the lymphatic cells of the immune system. Typically, lymphomas present as a solid tumor of lymphoid cells. Treatment might involve chemotherapy and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage...
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 diagnosisDiagnosis
Diagnosis is the identification of the nature and cause of anything. Diagnosis is used in many different disciplines with variations in the use of logics, analytics, and experience to determine the cause and effect relationships...
. A biopsy
Biopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
is the ultimate method for diagnosis, but is not always possible. The disease can even occur with no painful symptoms at all.
LYMF and LAM are easily confused and must be differentiated for an accurate diagnosis. LYMF is frequently diagnosed in children nearing puberty. Although this disease is not limited to being identified in infants, young children, and adults of both genders, whereas LAM is primarily a disease found in women of child-bearing age.
Common co-morbidities
Lymphangiomatosis is sometimes confused with Gorham's diseaseGorham's disease
Gorham's disease is a rare congenital disorder characterized by proliferation of vascular channels that cause massive destruction of bone matrix.The disease was first described by Gorham et al...
(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. More recently, it has been effectively proven that Interferon Injections (Chemotherapy treatment)help slow the growth of these lymphatic masses.Prognosis
Both LYMF and LAM are have a common fatality rate, 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 not all patients 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.