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Medical management of tumors associated with Kasabach-Merritt phenomenon: an expert survey. J Pediatr Hematol Oncol 2013 Nov;35(8):618-22 PMID: 23774156

Pubmed ID

23774156

DOI

10.1097/MPH.0b013e318298ae9e

Abstract

Kasabach-Merritt phenomenon (KMP) is a rare consumptive coagulopathy characterized by profound thrombocytopenia and hypofibrinogenemia occurring in association with the vascular tumors kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). Treatment remains challenging without consensus on the optimal medical management. The authors compiled expert opinions regarding management to establish treatment recommendations. Twenty-seven vascular anomalies centers in the United States and Canada were surveyed using 2 representative cases of KHE/TA with and without KMP. Overall response rate was 92% (25/27) with 88% completion (24/27). Most sites (23/25; 92%) do not have a standard of practice for management. The most frequent initial therapy for KHE+KMP was a combination of systemic corticosteroids and vincristine (VCR) (12/24 centers; 50%) followed by corticosteroids alone (29%). Second-line treatments were VCR (38%), rapamycin (21%), and propranolol (21%). Management of KHE/TA without KMP was variable; initial treatments included systemic corticosteroids (8/24; 33%) alone or with VCR (9/24; 38%), monitoring without medication (33%), VCR (8%), propranolol (8%), aspirin (4%), and rapamycin (4%). This survey highlights certain trends in the management of KMP-associated tumors, without standard protocols and consensus.

Author List

Tlougan BE, Lee MT, Drolet BA, Frieden IJ, Adams DM, Garzon MC



Scopus

2-s2.0-84891686257   28 Citations

MESH terms used to index this publication - Major topics in bold

Adrenal Cortex Hormones
Antineoplastic Agents
Data Collection
Hemangioendothelioma
Hemangioma
Humans
Kasabach-Merritt Syndrome
Practice Patterns, Physicians'
Propranolol
Sarcoma, Kaposi
Skin Neoplasms
Vasodilator Agents
Vincristine
jenkins-FCD Prod-332 f92a19b0ec5e8e1eff783fac390ec127e367c2b5