Kasabach-Merritt Syndrome: Causes, Symptoms, Diagnosis, and Treatment

Kasabach-Merritt Syndrome: Causes, Symptoms, Diagnosis, and Treatment

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Focused Health Topics
Contributed byAlexander Enabnit+2 moreJun 22, 2023

Introduction

Kasabach-Merritt syndrome (KMS) is a rare and potentially life-threatening condition that involves the formation of a vascular tumor with severe thrombocytopenia and coagulopathy. In this article, we will discuss the causes, symptoms, diagnosis, and treatment options for Kasabach-Merritt syndrome.

Causes: 

Kasabach-Merritt syndrome is caused by the abnormal growth of a vascular tumor, which can be either benign or malignant. The exact cause of this tumor growth is not well understood, but it is thought to be related to the abnormal proliferation of blood vessels. Other factors that may contribute to the development of Kasabach-Merritt syndrome include:

  • Infection: In some cases, the growth of the vascular tumor may be triggered by an infection.
  • Trauma: Trauma or injury to the affected area may also contribute to the development of Kasabach-Merritt syndrome.

Symptoms:

The symptoms of Kasabach-Merritt syndrome can vary depending on the size and location of the vascular tumor. Some of the common symptoms include:

  • Rapidly growing mass or lump on the skin, which can be painful, discolored, and warm to the touch
  • Severe thrombocytopenia and coagulopathy, which can cause bleeding, bruising, and petechiae (small red or purple spots on the skin)
  • Anemia, which can cause fatigue, weakness, and shortness of breath
  • Fever and malaise

Diagnosis:

The diagnosis of Kasabach-Merritt syndrome involves a combination of physical examination, medical history, and laboratory tests. Some of the tests that may be conducted to diagnose Kasabach-Merritt syndrome include:

  • Imaging tests: Imaging tests, such as ultrasound, CT scan, or MRI, may be done to visualize the vascular tumor and determine its size and location.
  • Blood tests: Blood tests may be done to check for thrombocytopenia, coagulopathy, and anemia.
  • Biopsy: A sample of the affected tissue may be taken and examined under a microscope to determine the type of tumor.

Treatment:

The treatment of Kasabach-Merritt syndrome depends on the severity of the condition and the overall health of the individual. Some of the treatment options for Kasabach-Merritt syndrome include:

  • Medical management: Medical management may be used to stabilize the individual's condition and control bleeding and coagulopathy. This may involve transfusion of blood products, such as platelets, fresh frozen plasma, and red blood cells.
  • Surgical removal: Surgical removal of the vascular tumor may be necessary if it is causing significant bleeding, obstructing nearby organs, or affecting the individual's quality of life.
  • Embolization: Embolization involves blocking the blood vessels that supply the vascular tumor, which can help reduce its size and prevent bleeding.
  • Radiation therapy: Radiation therapy may be used to shrink the vascular tumor and prevent its growth.

Conclusion:

Kasabach-Merritt syndrome is a rare and potentially life-threatening condition that involves the formation of a vascular tumor with severe thrombocytopenia and coagulopathy. Symptoms include a rapidly growing mass or lump on the skin, bleeding, and anemia. Diagnosis involves a combination of physical examination, medical history, and laboratory tests. Treatment options include medical management, surgical removal, embolization, and radiation therapy.

Hashtags: #KasabachMerrittSyndrome #VascularTumor #Thrombocytopenia #Coagulopathy #Bleeding #Anemia #Diagnosis #Treatment

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On the Article

Krish Tangella MD, MBA picture
Approved by

Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team
Alexander Enabnit picture
Author

Alexander Enabnit

Senior Editorial Staff
Alexandra Warren picture
Author

Alexandra Warren

Senior Editorial Staff

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