Which are the symptoms of Kasabach-Merritt syndrome?

Symptoms of Kasabach-Merritt Syndrome

Kasabach-Merritt syndrome is a rare and potentially life-threatening condition characterized by the presence of a vascular tumor, known as a hemangioma, along with severe thrombocytopenia (low platelet count) and consumption coagulopathy (abnormal blood clotting). This syndrome primarily affects infants and young children, although it can occur in individuals of any age.

Hemangioma: The hallmark feature of Kasabach-Merritt syndrome is the presence of a large, rapidly growing hemangioma. These tumors are composed of blood vessels and can appear as a raised, red or purple lesion on the skin or internal organs. Hemangiomas associated with this syndrome tend to be larger and more aggressive than typical hemangiomas.

Thrombocytopenia: One of the key symptoms of Kasabach-Merritt syndrome is a significant decrease in platelet count. Platelets are essential for blood clotting, and low levels can lead to excessive bleeding and bruising. Thrombocytopenia in this syndrome is often severe and can be life-threatening if not promptly addressed.

Consumption Coagulopathy: Kasabach-Merritt syndrome is also characterized by consumption coagulopathy, which refers to the abnormal consumption of clotting factors in the blood. This can result in a prolonged clotting time, leading to excessive bleeding and difficulty in controlling bleeding. Consumptive coagulopathy can further exacerbate the risk of hemorrhage in individuals with this syndrome.

Enlarged Organs: In some cases, the hemangioma associated with Kasabach-Merritt syndrome may develop internally, affecting organs such as the liver, spleen, or intestines. This can lead to organ enlargement, causing abdominal pain, discomfort, and potential complications depending on the affected organ.

Disseminated Intravascular Coagulation (DIC): In severe cases, Kasabach-Merritt syndrome can progress to disseminated intravascular coagulation, a condition characterized by widespread blood clotting and subsequent bleeding. DIC can further contribute to the complications associated with this syndrome.

Other Symptoms: Depending on the location and size of the hemangioma, individuals with Kasabach-Merritt syndrome may experience additional symptoms. These can include skin ulceration, difficulty breathing or swallowing (if the tumor affects the airway or digestive tract), high-output heart failure (if the tumor involves the heart), or neurological symptoms (if the tumor affects the brain).

It is important to note that the severity and specific symptoms of Kasabach-Merritt syndrome can vary among individuals. Prompt diagnosis and appropriate management are crucial to prevent complications and improve outcomes.