About Kasabach-Merritt Syndrome

What is Kasabach-Merritt Syndrome?

Kasabach-Merritt Syndrome (KMS) is a rare disorder characterized by the formation of a large, blood-filled mass (hematoma) in the body. It is caused by a combination of a platelet disorder and a vascular abnormality. Symptoms may include bleeding, bruising, and anemia. Treatment typically involves medications to reduce the size of the hematoma and to prevent further bleeding.

What are the symptoms of Kasabach-Merritt Syndrome?

The main symptoms of Kasabach-Merritt Syndrome (KMS) include:

-Large, rapidly growing, non-malignant blood vessel tumors (hemangiomas)
-Low platelet count (thrombocytopenia)
-Anemia (low red blood cell count)
-Enlarged spleen and/or liver
-High levels of fibrinogen in the blood
-High levels of certain proteins in the blood
-Fever
-Failure to thrive
-Bruising
-Bleeding
-Jaundice
-Lethargy
-Shortness of breath
-Swollen lymph nodes
-Skin ulcers

What are the causes of Kasabach-Merritt Syndrome?

Kasabach-Merritt Syndrome (KMS) is a rare disorder caused by a combination of a vascular malformation and a consumptive coagulopathy. The exact cause of KMS is unknown, but it is believed to be caused by an abnormal interaction between the vascular malformation and the body's clotting system. It is thought that the vascular malformation causes an increased production of platelets, which leads to a decrease in the body's ability to clot. This can lead to excessive bleeding and clotting in the affected area.

What are the treatments for Kasabach-Merritt Syndrome?

The main treatments for Kasabach-Merritt Syndrome are:

1. Surgery: Surgery is often used to remove the large blood vessels that are trapping the platelets and causing the syndrome.

2. Blood transfusions: Blood transfusions can be used to replace the platelets that are being trapped and destroyed.

3. Medications: Medications such as corticosteroids, vincristine, and interferon can be used to reduce the size of the blood vessels and reduce the platelet destruction.

4. Platelet transfusions: Platelet transfusions can be used to replace the platelets that are being destroyed.

5. Radiation therapy: Radiation therapy can be used to shrink the large blood vessels and reduce the platelet destruction.

What are the risk factors for Kasabach-Merritt Syndrome?

The exact cause of Kasabach-Merritt Syndrome is unknown, but certain risk factors have been identified. These include:
* Having a large, deep vascular malformation (such as a hemangioma)
* Having a family history of Kasabach-Merritt Syndrome
* Being a female
* Being of Asian or Hispanic descent
* Having a low birth weight
* Having a weakened immune system

Is there a cure/medications for Kasabach-Merritt Syndrome?

Yes, there are treatments available for Kasabach-Merritt Syndrome. Treatment typically involves a combination of medications, such as corticosteroids, anticoagulants, and immunosuppressants, as well as surgical interventions. In some cases, chemotherapy may also be used. It is important to work with a healthcare provider to determine the best treatment plan for each individual case.