Kasabach–Merritt syndrome (KMS) is a potentially life-threatening coagulopathy characterized by enlarging hemangioma with severe thrombocytopenia.[1] KMS. Kasabach-Merritt syndrome is characterised by the combination of rapidly growing vascular tumour, thrombocytopenia, microangiopathic haemolytic anaemia. [2] Thereafter, the association of a capillary hemangioma and thrombocytopenia was labeled Kasabach-Merritt syndrome (the name was later changed to KMP).

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Successful treatment of kaposiform hemangioendothelioma and tufted angioma with vincristine. Her vital signs and oxygen saturation were normal.

Effective therapy of a vascular tumor of infancy with vincristine. The platelet count improved and the steroids were continued for 3 months.

Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: However, while the patient was there, she developed severe respiratory distress with inspiratory stridor and desaturation. In other projects Wikimedia Commons. His serum electrolytes synvrome renal function tests were within normal limits. A clinical diagnosis of hemangioma involving the right parotid extending on to the right temporal area was made.

Box Bethesda, MD Phone: On routine physical examination, she was ill looking, pale, not cyanosed or jaundiced. All subjects with KMP had profound msrritt and hypofibrinogenemia with elevated fibrin split products D-dimerssuggestive of an active consumptive coagulopathy.


Kasabach-merritt syndrome.

It can be achieved by different techniques: No arteriovenous malformation was noted in brain or other body parts. Areas of petechiae tiny red dots can appear around the lesion or on other parts of the body. Ultrasound and computed tomography CT of brain and abdomen were normal.

N Engl J Med.

Kasabach – Merritt syndrome: A case report

Patients diagnosed with these conditions need to be treated at multidisciplinary vascular anomaly centers. The baby was subsequently discharged on oral steroids and propranolol. Considering possibility of steroid induced cholestasis, tapering of steroids was started.

Propranolol should mmerritt gradually tapered over a period of 2 weeks. Please review our privacy policy. The blood clotting disorder results from platelets and other clotting factors of the blood being used up within the tumor.

Kasabach-Merritt phenomenon – NORD (National Organization for Rare Disorders)

Interferon alpha has been used in some of the studies as second line therapy kasagach failure of steroids. Neurotoxicity is the dose-limiting side effect of vincristine. Adverse effects of interferon alpha-2b include constitutional symptoms e. A full-term male baby was referred at 4 days of age with a large CH on the left side of the chest wall.


Kasabach–Merritt syndrome with large cutaneous vascular tumors

D-dimer was positive and suggestive of consumptive coagulopathy. Self-skin examination New smartphone apps to check your skin Learn more Sponsored content.

When evaluating a patient with these types of malformations, one must also consider syndromes associated with vascular malformations, such as Klippel-Trenaunay-Weber syndrome and Sturge-Weber syndrome. Diagnosis of KMS is made based on the constellation of a vascular lesion, thrombocytopenia, consumptive coagulopathy, and microangiopathic hemolytic anemia. Currently, there are no known treatment guidelines.

Capillary hemangioma with extensive purpura: If the vital signs and glucose levels remain normal, the dose is incrementally doubled to a maximum of 0. This page was last edited on 13 Novemberat