| Autor: |
Singh, Sanjay, Bhari, Neetu, Jassi, Rubina |
| Předmět: |
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| Zdroj: |
Indian Journal of Paediatric Dermatology; Oct-Dec2019, Vol. 20 Issue 4, p295-301, 7p |
| Abstrakt: |
Kasabach-Merritt Phenomenon (KMP) is a potentially life-threatening condition characterised by thrombocytopenic consumptive coagulopathy. KMP is almost exclusively associated with two uncommon vascular tumours, Kaposiform haemangioendotheliomas (KHE) and tufted angiomas (TA). It is clinically characterized by a rapid increase in the size of the pre-existing vascular plaque and deranged blood coagulation profiles. Early recognition and treatment is of crucial importance. Hemodynamic stability should be achieved as soon as possible with the use of fresh frozen plasma infusions. Surgical excision and embolization, when feasible, are the line of treatment. Oral prednisolone with or without vincristine are most commonly used and effective treatment. Oral sirolimus has shown its efficacy and safety in recent reports and-series. Propranolol and anti-platelet drugs are commonly used as a second-line therapy. Other drugs and combination therapies are used in non-responsive recalcitrant cases. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
| Externí odkaz: |
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