Kasabach–Merritt phenomenon
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 charac...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2019-01-01
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| Series: | Indian Journal of Paediatric Dermatology |
| Subjects: | |
| Online Access: | http://www.ijpd.in/article.asp?issn=2319-7250;year=2019;volume=20;issue=4;spage=295;epage=301;aulast=Singh |
| _version_ | 1819014603029348352 |
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| author | Sanjay Singh Neetu Bhari Rubina Jassi |
| author_facet | Sanjay Singh Neetu Bhari Rubina Jassi |
| author_sort | Sanjay Singh |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-21T02:18:28Z |
| format | Article |
| id | doaj.art-9183d0321f354aa589b6cd2626e8a524 |
| institution | Directory Open Access Journal |
| issn | 2319-7250 |
| language | English |
| last_indexed | 2024-12-21T02:18:28Z |
| publishDate | 2019-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Paediatric Dermatology |
| spelling | doaj.art-9183d0321f354aa589b6cd2626e8a5242022-12-21T19:19:12ZengWolters Kluwer Medknow PublicationsIndian Journal of Paediatric Dermatology2319-72502019-01-0120429530110.4103/ijpd.IJPD_2_18Kasabach–Merritt phenomenonSanjay SinghNeetu BhariRubina JassiKasabach-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.http://www.ijpd.in/article.asp?issn=2319-7250;year=2019;volume=20;issue=4;spage=295;epage=301;aulast=SinghKasabach–Merritt phenomenonthrombocytopenic consumptive coagulopathyKaposiform hemangioendotheliomatufted angioma |
| spellingShingle | Sanjay Singh Neetu Bhari Rubina Jassi Kasabach–Merritt phenomenon Indian Journal of Paediatric Dermatology Kasabach–Merritt phenomenon thrombocytopenic consumptive coagulopathy Kaposiform hemangioendothelioma tufted angioma |
| title | Kasabach–Merritt phenomenon |
| title_full | Kasabach–Merritt phenomenon |
| title_fullStr | Kasabach–Merritt phenomenon |
| title_full_unstemmed | Kasabach–Merritt phenomenon |
| title_short | Kasabach–Merritt phenomenon |
| title_sort | kasabach merritt phenomenon |
| topic | Kasabach–Merritt phenomenon thrombocytopenic consumptive coagulopathy Kaposiform hemangioendothelioma tufted angioma |
| url | http://www.ijpd.in/article.asp?issn=2319-7250;year=2019;volume=20;issue=4;spage=295;epage=301;aulast=Singh |
| work_keys_str_mv | AT sanjaysingh kasabachmerrittphenomenon AT neetubhari kasabachmerrittphenomenon AT rubinajassi kasabachmerrittphenomenon |