What is Kasabach-Merritt Syndrome?

Kasabach-Merritt syndrome (KMS) is a rare, life-threatening condition involving underdeveloped blood vessels which form a large growth (called either a Kaposiform hemangioendothelioma or tufted angioma) and may interfere with blood clotting. Kasabach-Merritt syndrome is also known as hemangioma thrombocytopenia syndrome.

What are the symptoms of KMS?

Symptoms may include:

  • Reddish-brown, fast-growing growths (also called hemangiomas) can be found on the trunk, arms, shoulders, legs, neck, or face and can also occur in internal organs such as the liver, spleen, or gastrointestinal tract
  • Pain within the growths
  • Prolonged bleeding
  • Frequent bruising
  • How is KMS diagnosed?

A full physical exam and blood tests will be conducted. Imaging like traditional X-rays; angiography, which uses an injectable dye and X-rays; computed tomography (CT) scan, which produces a three dimensional view of the scanned area; and magnetic resonance imaging (MRI) which uses powerful magnets and radio waves to create detailed organ and tissue mapping. In some cases, a biopsy of the tumor may be needed.

How is KMS treated?

If a child with KMS has low levels of platelets (disc-shaped structures in the blood that help with clotting), he or she may need to be admitted to the hospital for a platelet and/or a plasma transfusion. In some cases, the growths and the clotting problem will not both be resolved; for example, the clotting problem may be solved but the growth could remain. Medications or surgery may treat the growth.

About treatment for KMS at Children's

Kasabach-Merritt syndrome is commonly treated through Children's Vascular Anomalies Center, which is located on our Minneapolis campus. By coordinating experts from several pediatric disciplines into a single visit, families experience a new level of convenience in an environment focused exclusively on pediatrics. Every child is assigned a pediatric nurse case manager who coordinates all treatment and communication between care providers and the family.

Contact Us

For consultation, referral, or an appointment, call the Vascular Anomalies Center at 612-813-7100.