Kasabach-Merritt syndrome
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Kasabach-Merritt syndrome; thrombocytopenia-hemangioma syndrome
Overview
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Kasabach-Merritt Syndrome (KMS) is a rare disease, usually of infants, in which a vascular tumor leads to decreased platelet counts and sometimes other bleeding problems,[1] which can be life-threatening.[2] It is also known as hemangioma thrombocytopenia syndrome.
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Historical Perspective
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References
Pathophysiology
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Pathophysiology
KMS is usually caused by a hemangioendothelioma or other vascular tumor, often present at birth.[1][2] Although these tumors are relatively common, it is rare for them to cause KMS.
When these tumors are large or are growing rapidly, sometimes they can trap platelets, causing severe thrombocytopenia. The combination of vascular tumor and consumptive thrombocytopenia defines KMS. Tumors can be found in the trunk, upper and lower extremities, retroperioneum, and in the cervical and facial areas.
This consumptive coagulopathy also uses up clotting factors, such as fibrinogen which may worsen bleeding. The coagulopathy can progress to disseminated intravascular coagulation and even death.
References
- ↑ Enjolras O, Wassef M, Mazoyer E, Frieden I, Rieu P, Drouet L, Taïeb A, Stalder J, Escande J (1997). “Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas”. J Pediatr. 130 (4): 631–40. PMID 9108863.
- ↑ el-Dessouky M, Azmy A, Raine P, Young D (1988). “Kasabach-Merritt syndrome”. J Pediatr Surg. 23 (2): 109–11. PMID 3278084.
Causes
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Differentiating Kasabach-Merritt syndrome from other Diseases
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Epidemiology and Demographics
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Natural History, Complications and Prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Natural History
Complications
They are at risk of bleeding complications including intracranial hemorrhage. The thrombocytopenia and coagulopathy are managed with platelet transfusions and fresh frozen plasma, although caution is needed due to the risk of fluid overload and heart failure from multiple transfusions. The possibility of disseminated intravascular coagulation, a dangerous and difficult-to-manage condition, is concerning. Anticoagulant and antiplatelet medications can be used after careful assessment of the risks and benefits.
Prognosis
Patients with KMS can be extremely ill and may need intensive care. KMS has a mortality rate of about 30%. For patients that survive the acute disease, supportive care may be required through a gradual recovery.
Furthermore, patients may need care from a dermatologist or plastic surgeon for residual cosmetic lesions. On long-term followup, most patients have skin discoloration and/or mild disfiguration from the dormant tumor.[1]
References
- ↑ Enjolras O, Mulliken J, Wassef M, Frieden I, Rieu P, Burrows P, Salhi A, Léauté-Labrèze C, Kozakewich H (2000). “Residual lesions after Kasabach-Merritt phenomenon in 41 patients”. J Am Acad Dermatol. 42 (2 Pt 1): 225–35. PMID 10642677.
Diagnosis
Diagnosis
History and Symptoms | Physical Examination | Laboratory Findings | CT | MRI | Echcardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies
Treatment
Treatment
Medical Therapy | Surgery | Cost-Effectiveness of Therapy | Future or Investigational Therapies
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