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Kasabach-Merritt syndrome: a case review
1Department of Pediatrics, Tufts University School of Medicine, USA.
Neonatal Network : NN
|April 4, 1998
Summary
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.