Kaposiform lymphangiomatosis

What is the cause of kaposiform lymphngiomatosis?

The cause of kaposiform lymphangiomatosis (KLA) is unknown. KLA is the result of an abnormality in formation of the lymph system during fetal development.

Last updated on 05-01-20

How is kaposiform lymphangiomatosis diagnosed?

The diagnosis of kaposiform lymphangiomatosis (KLA) is based on the symptoms and the distinct features of the tumors formed in KLA. A small sample of tumor tissue examined under a microscope (biopsy) can help confirm the diagnosis. Tumor genetic testing may also show specific genetic changes that can help with the diagnosis.

Last updated on 05-01-20

Is kaposiform lymphangiomatosis inherited?

Kaposiform lymphangiomatosis is not thought to be inherited in families.

Last updated on 05-01-20

What is the long-term outlook for people with kaposiform lymphangiomatosis?

The duration and outcome of a rare disease like kaposiform lymphangiomatosis (KLA) is influenced by many factors. These include the severity of the symptoms, the availability of treatment, other medical conditions and lifestyle factors.

People with KLA may begin to have symptoms in childhood. The first symptoms may include a dry cough and body pain. KLA is a progressive condition that gets worse over time. Some of the symptoms such as the build-up of fluid around the lungs and heart and abnormal bleeding may be life threatening.

Last updated on 05-01-20

How might kaposiform lymphangiomatosis be treated?

There is no specific treatment for kaposiform lymphangiomatosis (KLA). Treatment is based on managing the symptoms and controlling the growth of abnormal lymph vessels. Treatment options may include surgical procedures to drain excess fluid, medications to help control bleeding, and chemotherapy to help stabilize the condition.

Last updated on 05-01-20

Name: Lymphangiomatosis & Gorham's Disease Alliance 19919 Villa Lante Place
Boca Raton, FL, 33434, United States
Phone: +1-561-441-9766 Toll Free: 1-844-588-5771 Email: info@lgdalliance.org Url: https://www.lgdalliance.org
Barclay SF Inman KW, Luks VL, McIntyre JB, Al-Ibraheemi A, Church AJ, Perez-Atayde AR, et al. A somatic activating NRAS variant associated with kaposiform lymphangiomatosis. Genet Med. Dec 13, 2018; epub. Reference Link Adams DM, Ricci KW. Vascular Anomalies: Diagnosis of Complicated Anomalies and New Medical Treatment Options Hematol Oncol Clin North Am. Jun 2019; 33(3). 455-470. Reference Link

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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