Abstract
The Kasabach-Merritt syndrome consists of a cavernous hemangioma with internal intravascular coagulation associated with a microangiopathic hemolytic anemia. In some patients, activation of clotting and fibrinolysis leads to thrombocytopenia and coagulopathy. Often these lesions are cutaneous and are readily detected by clinical evaluation. Rarely these lesions may be occult, involving the liver, spleen, or even a soft tissue mass simulating a malignant tumor. In those patients in whom the lesion is readily apparent, there is no need for a diagnostic procedure although angiography is occasionally utilized for confirmation. The use of Technetium (Tc)-99m labeled red blood cells (RBC) provides a total body survey in those individuals in whom an occult hemangioma is suspected and establishes the diagnosis in those patients in whom a cutaneous lesion is present. The patient's own red cells are labeled in vitro and reinjected allowing excellent imaging. This procedure is relatively noninvasive and has a low radiation burden. We report the use of this technique in five patients presenting with Kasabach-Merritt syndrome. Three of these patients had occult or unsuspected lesions. No other imaging modality provides this graphic demonstration of these lesions. The technique of this procedure and illustrative cases will be presented.
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Loh, W., Miller, J. & Gomperts, E. THE DETECTION OF OCCULT HEMANGIOMAS PRODUCING THE KASABACH-MERRITT SYNDROME UTILIZING Tc-99m LABELED RED BLOOD CELLS. Pediatr Res 21 (Suppl 4), 301 (1987). https://doi.org/10.1203/00006450-198704010-00803
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DOI: https://doi.org/10.1203/00006450-198704010-00803