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

Cashell, Jamie; Smink, Gayle; Helm, Klaus F.; Xavier, Frederico

doi:10.1002/pbc.27305

Cited by 18 publications

(14 citation statements)

References 21 publications

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“…No hay reportes de afectos adversos graves. Aunque se obtuvo una respuesta positiva con toxicidad limitada en los casos publicados, no existe una estandarización de los criterios de respuesta ni de toxicidad (Tabla 1) [29][30][31][32][33][34][35][36][37] . Los HEK son tumores vasculares infrecuentes y localmente agresivos en la edad pediátrica 1 .…”

Section: Discussionunclassified

Hemangioendotelioma kaposiforme refractario en población pediátrica: reporte de caso y revisión de la literatura

Gómez-Villegas¹,

Pérez-Téllez²,

Ochoa-Gaviria³

et al. 2021

BMHIM

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Introducción: El hemangioendotelioma kaposiforme (HEK) es un tumor vascular poco frecuente caracterizado por una invasión local agresiva y un síndrome de atrapamiento de plaquetas conocido como fenómeno de Kasabach-Merritt. Aunque muchos casos de HEK se tratan con éxito con control local o quimioterapia de baja intensidad, otros son resistentes y se cuenta con pocas opciones terapéuticas. El objetivo de este reporte es mostrar la experiencia del tratamiento con sirolimus por vía oral en un paciente pediátrico con HEK asociado a fenómeno de Kasabach-Merritt refractario al tratamiento de primera línea, quien mostró excelente respuesta al tratamiento. Caso clínico: Paciente de sexo masculino de 3 meses con un HEK refractario al manejo de primera línea (corticoides, propranolol, vincristina), sin posibilidad de hacer control local, por lo que se decide terapia combinada con sirolimus, presentando control local y resolución de la coagulopatía desde la primera semana de iniciado el manejo y con resolución de la malformación vascular después de 12 meses de seguimiento. Conclusiones: Aunque no existen pautas claras para el tratamiento del HEK refractario en la edad pediátrica, la evidencia actual demuestra que el sirolimus es un medicamento eficaz que puede ser considerado como opción terapéutica de primera línea en estos pacientes.

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Section: Discussionunclassified

Hemangioendotelioma kaposiforme refractario en población pediátrica: reporte de caso y revisión de la literatura

Gómez-Villegas¹,

Pérez-Téllez²,

Ochoa-Gaviria³

et al. 2021

BMHIM

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“…Although the effects of propranolol in the treatment of infantile hemangiomas have been demonstrated, the effects of pro pranolol monotherapy on KMP are limited 17) . Most recently, there was a case report of successful treatment using predni solone, vincristine, and sirolimus 18) . Percutaneous transar terial embolization could be another therapeutic option.…”

Section: Discussionmentioning

confidence: 99%

Successful Management of Visceral Kaposiform Hemangioendothelioma with Kasabach‐Merritt Phenomenon Using Corticosteroids and Vincristine

2020

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Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular neopla sm that often develops a coagulopathy known as KasabachMerritt phenomenon (KMP). Visceral involvement denotes a poor prognosis. We report a case of visceral KHE with KMP successfully treated with corticosteroids and vincristine. The infant had been born vaginally at 34 weeks 6 days' gestation, weighing 2,360 g. He was admi tted for the management of respiratory failure. Blood tests showed anemia and thrombo cytopenia 1 hour after delivery. Additional blood tests revealed a prothrombin time of 12.1 seconds, activated partial thromboplastin time of 60.6 seconds, fibrinogen le vels of 72.4 mg/dL, and Ddimer levels >3,200 ng/mL. Despite supportive measures and daily transfusions, the clinical condition and coagulopathy gradually worsened. Renal ultrasonography performed to find the origin of the coagulopathy revealed an echogenic mass measuring >3 cm in the abdominal cavity. A magnetic resonance imag ing scan showed an illmarginated, infiltrative mass like lesion in the right anteromedial and posteromedial perirenal space that was hypointense with mild enhance ment on T1 and T2weighted images. Large vascular tumors adherent to other visceral organs were noted during exploratory laparotomy but could not be resected. Treatment with methylprednisolone was ineffective. Vincristine was administered weekly from the 17th hospital day, and the coagulation profile showed gradual impro vement after its initia tion. Intravenous methylprednisolone was switched to oral prednisolone on the 57th hospital day. He was discharged on the 73rd hospital day and continued vincristine treatment every 2 weeks and oral prednisolone administration as an outpatient treat ment for 8 weeks. He remained symptomfree at the 39month followup.

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“…In addition, the drug therapy for KHE using angiogenesis inhibitor sirolimus targeting the mammalian target of rapamycin (mTOR) [13][14][15] , have been shown to be efficacious. While prednisolone, vincristine and sirolimus showed some effectiveness in decreasing the tumor size 16,17 , can cause side effects such as abdominal pain, elevations of aspartate aminotransferase (AST) and alanine aminotransferase (ALT), opportunistic infection, loss of appetite, etc 18 . Moreover, there are case reports of visceral KHE not responding to sirolimus 19,20 .…”

Section: Discussionmentioning

confidence: 99%

Curative effect of surgical resection in the treatment of Kaposiform hemangioendothelioma

Zuo

Guo

Wang

et al. 2020

Preprint

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Background The clinical features and surgical outcomes of patients with Kaposiform hemangioendothelioma (KHE) is not fully clear. The purpose of this study is to better understand the therapeutic effect and hematological indices of surgical therapy for KHE. Procedure A retrospective study was conducted to review the medical documents of 43 patients with KHE who were treated with surgical resection at our hospital between February 2016 and November 2019. Multiple anatomical sites were involved. The curative effect was evaluated according to the tumor volume shrinkage, color shade, and blood examinations including platelet count, hemoglobin level, red blood cell number, clotting time and D-dimer concentration. Results For all cases underwent surgical treatment, the curative effects accounted for 100%. At the end of the treatment, 9 children were followed up for 6 months, and no evidence of the recurrence of KHE was not found in any case. The number of red blood cells and clotting time were maintained at normal levels during different stage of treatment. The concentrations of hemoglobin and fibrinogen and the average number of platelets increased significantly after surgical treatment. The concentrations of D-dimer were much higher than normal value during admission with a high variability, and significantly decreased after surgery treatment. Further analysis found that the changing trends of D-dimer was positively correlated with fibrinogen protein or platelet number, but not clotting time. Conclusions The surgical treatment method is highly effective and of great significance for KHE therapy in pediatric clinic.

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

Cited by 18 publications

References 21 publications

Hemangioendotelioma kaposiforme refractario en población pediátrica: reporte de caso y revisión de la literatura

Hemangioendotelioma kaposiforme refractario en población pediátrica: reporte de caso y revisión de la literatura

Successful Management of Visceral Kaposiform Hemangioendothelioma with Kasabach‐Merritt Phenomenon Using Corticosteroids and Vincristine

Curative effect of surgical resection in the treatment of Kaposiform hemangioendothelioma

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