Kasabach-Merritt syndrome: a case review.
Hemangiomas are common newborn vascular tumors occurring in up to 2.5 percent of newborns. Most are benign and 70 to 80 percent regress by age seven. Some hemangiomas are life threatening--1 in 300 is associated with coagulopathy. Kasabach-Merritt syndrome (KMS), associated with hemangioma, disseminated intravascular coagulopathy, microangiopathic anemia, and thrombocytopenia, can have profound sequelae. Morbidity and mortality are influenced by the anatomic location and size of the hemangioma. Untreated KMS has a 10-37 percent mortality rate. Bleeding secondary to the consumptive coagulopathy is the primary cause of death in these patients. As of 1997, there have been approximately 205 cases of KMS reported in the literature. This case study discusses the pathophysiology, clinical manifestations, diagnostics, treatment modalities, differential diagnosis, and psychosocial considerations of KMS.
Szlachetka
D M
DM
Department of Pediatrics, Tufts University School of Medicine, USA.
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003131:Combined Modality Therapy; D004211:Disseminated Intravascular Coagulation / Q000150:complications* / Q000628:therapy; D006391:Hemangioma / Q000150:complications* / Q000628:therapy; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D017713:Platelet Transfusion; D013577:Syndrome; D013921:Thrombocytopenia / Q000150:complications* / Q000628:therapy
D000893:Anti-Inflammatory Agents; D008775:Methylprednisolone