Authors: A. Kumar, M.D., V. Dimov, M.D., The Cleveland Clinic
An one-year old boy presented to the hospital with right upper arm swelling since birth (click to enlarge the picture) and a low platelet count done by the primary care physician. No other skin lesions were found. CT of the chest and abdomen were negative.
The child has had a normal development.
Steroids were started for suspected immune thrombocytopenia (ITP) but despite a 10-day course the platelet count went down to 1,500.
Fig. 1: Hemangioma of right upper arm
What do you think is going on?
The boy has a hemangioma and thrombocytopenia. Kasabach-Merritt syndrome presents with this combination of symptoms.
What would you do?
Confirm platelet count.
Consult pediatric hematology and vascular surgery.
The pediatric hematologist recommended a trial of Vincristine to shrink the hemangioma. The vascular surgeon recommended embolization of the hemangioma if there is no effect from Vincristin therapy.
What did we learn from this case?
Suspect Kasabach-Merritt syndrome in patients with hemangioma and thrombocytopenia. Thrombocytopenia is caused by sequestration and destruction of platelets in a large cavernous hemangioma. The syndrome is usually seen in infants but can also be rarely observed in adults.
No single treatment approach is consistently effective.
Kasabach-Merritt Syndrome. eMedicine, topic 1234.
Kasabach-Merritt Syndrome. eMedicine, topic 1221.
Propranolol for Severe Hemangiomas of Infancy. NEJM, 06/2008.