Kaposiform hemangioendothelioma with Kasabach–Merritt phenomenon in an infant: Successful treatment with prednisolone, vincristine, and addition of sirolimus

Jamie Cashell, Gayle Smink, Klaus Helm, Frederico Xavier

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

A full-term newborn with kaposiform hemangioendothelioma (KHE) affecting the right thigh with thrombocytopenia due to Kasabach–Merritt phenomenon (KMP) was referred to our center. After biopsy, he rapidly evolved to severe thrombocytopenia and severe coagulopathy. Standard therapy was initiated with prednisolone and vincristine. His coagulopathy worsened to life-threatening hemorrhage necessitating aggressive blood products replacement. Sirolimus was added; he became transfusion independent with no further bleeding and reduction in tumor size. Addition of sirolimus to treatment of vascular anomalies with hemostatic complications should be considered as part of early treatment for patients with KMP/KHE.

Original languageEnglish (US)
Article numbere27305
JournalPediatric Blood and Cancer
Volume65
Issue number12
DOIs
StatePublished - Dec 1 2018

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Vincristine
Sirolimus
Prednisolone
Thrombocytopenia
Hemorrhage
Hemostatics
Thigh
Blood Vessels
Therapeutics
Newborn Infant
Biopsy
Kaposiform Hemangioendothelioma
Neoplasms

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

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title = "Kaposiform hemangioendothelioma with Kasabach–Merritt phenomenon in an infant: Successful treatment with prednisolone, vincristine, and addition of sirolimus",
abstract = "A full-term newborn with kaposiform hemangioendothelioma (KHE) affecting the right thigh with thrombocytopenia due to Kasabach–Merritt phenomenon (KMP) was referred to our center. After biopsy, he rapidly evolved to severe thrombocytopenia and severe coagulopathy. Standard therapy was initiated with prednisolone and vincristine. His coagulopathy worsened to life-threatening hemorrhage necessitating aggressive blood products replacement. Sirolimus was added; he became transfusion independent with no further bleeding and reduction in tumor size. Addition of sirolimus to treatment of vascular anomalies with hemostatic complications should be considered as part of early treatment for patients with KMP/KHE.",
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AU - Smink, Gayle

AU - Helm, Klaus

AU - Xavier, Frederico

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AB - A full-term newborn with kaposiform hemangioendothelioma (KHE) affecting the right thigh with thrombocytopenia due to Kasabach–Merritt phenomenon (KMP) was referred to our center. After biopsy, he rapidly evolved to severe thrombocytopenia and severe coagulopathy. Standard therapy was initiated with prednisolone and vincristine. His coagulopathy worsened to life-threatening hemorrhage necessitating aggressive blood products replacement. Sirolimus was added; he became transfusion independent with no further bleeding and reduction in tumor size. Addition of sirolimus to treatment of vascular anomalies with hemostatic complications should be considered as part of early treatment for patients with KMP/KHE.

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