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Kasabach-Merritt syndrome: a case review
1Department of Pediatrics, Tufts University School of Medicine, USA.
Abstract:
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.
Insights
Kasabach-Merritt syndrome (KMS) is a rare, life-threatening condition associated with hemangiomas, causing severe coagulopathy. Early diagnosis and treatment are crucial to reduce the high mortality rate linked to bleeding complications.
Area of Science:
- Pediatric Oncology
- Hematology
- Vascular Biology
Background:
- Hemangiomas are common benign vascular tumors in newborns, with most regressing spontaneously.
- A small percentage of hemangiomas are associated with life-threatening complications, including coagulopathy.
Observation:
- Kasabach-Merritt syndrome (KMS) is a severe complication characterized by hemangioma, disseminated intravascular coagulopathy, microangiopathic anemia, and thrombocytopenia.
- Morbidity and mortality in KMS are significantly influenced by hemangioma size and location.
- Untreated KMS carries a substantial mortality rate, primarily due to bleeding from consumptive coagulopathy.
Findings:
- This case study reviews the pathophysiology, clinical presentation, diagnostic approaches, and treatment strategies for KMS.
- The literature reports approximately 205 cases of KMS as of 1997.
- Bleeding secondary to consumptive coagulopathy is the leading cause of death in KMS patients.
Implications:
- Understanding KMS pathophysiology is vital for timely diagnosis and intervention.
- Effective management requires a multidisciplinary approach addressing both the hemangioma and coagulopathy.
- Further research is needed to improve outcomes and reduce the mortality associated with KMS.