Which statement about Kasabach-Merritt syndrome is true?

Which statement about Kasabach-Merritt syndrome is true?

• A. It involves thrombocytopenia due to sequestration within large hemangiomas
• B. It causes hepatocellular carcinoma
• C. It occurs in adults only
• D. It is unrelated to hepatic hemangiomas

Answer: (A)

Kasabach-Merritt syndrome is a consumptive coagulopathy that happens when a large vascular tumor traps and consumes platelets and clotting factors. The tumor acts like a sponge, sequestering platelets, which drops the platelet count, and it also uses up fibrinogen and other components of the coagulation system, leading to bleeding risk. This scenario is most often described in infants with sizable vascular tumors such as kaposiform hemangioendothelioma or large infantile hemangiomas, and sometimes involves hepatic or other visceral lesions. That mechanism explains why the statement about thrombocytopenia due to sequestration within large hemangiomas is true. The other statements don’t fit: it is not a cause of hepatocellular carcinoma; it does not occur only in adults; and while hepatic hemangiomas can be involved, the condition is not unrelated to hepatic hemangiomas—hepatic involvement can occur in this syndrome.

Kasabach-Merritt syndrome is a consumptive coagulopathy that happens when a large vascular tumor traps and consumes platelets and clotting factors. The tumor acts like a sponge, sequestering platelets, which drops the platelet count, and it also uses up fibrinogen and other components of the coagulation system, leading to bleeding risk. This scenario is most often described in infants with sizable vascular tumors such as kaposiform hemangioendothelioma or large infantile hemangiomas, and sometimes involves hepatic or other visceral lesions.

That mechanism explains why the statement about thrombocytopenia due to sequestration within large hemangiomas is true. The other statements don’t fit: it is not a cause of hepatocellular carcinoma; it does not occur only in adults; and while hepatic hemangiomas can be involved, the condition is not unrelated to hepatic hemangiomas—hepatic involvement can occur in this syndrome.