Clinical features of Kasabach-Merritt syndrome: an analysis of 16 neonates / 中国当代儿科杂志

OBJECTIVE@#To study the clinical features, treatment, and prognosis of neonates with Kasabach-Merritt syndrome (KMS), and to provide a reference for optimizing the diagnosis and treatment of this disease.@*METHODS@#A retrospective analysis was performed for the clinical and follow-up data of 16 neonates with KMS who were admitted to the Anhui Children's Hospital, Anhui Medical University, from January 2016 to December 2020.@*RESULTS@#Of the 16 neonates, there were 13 boys (81%) and 3 girls (19%), with an age of 1 hour to 10 days on admission. Among these neonates, 13 (81%) had cutaneous hemangioma (2 in the head and face, 5 in the trunk, and 6 in the extremities) and 3 (19%) had liver hemangioma. The main clinical manifestations of bleeding tendency and scattered petechiae and ecchymosis were observed in 10 neonates (62%). All the 16 neonates had varying degrees of thrombocytopenia and coagulation disorders. They all received glucocorticoid treatment after admission and 7 (44%) of them had response, among whom 4 experienced recurrence. Among the neonates with no response to glucocorticoid treatment, 3 received sirolimus treatment, among whom 1 had the tumor volume reduced by 58.8% after 4 weeks of treatment, with platelet count and coagulation function returning to normal, while 2 had no significant reduction in tumor volume or significant increase in platelet count and achieved a tumor volume reduced by (43.7±0.4)% after 4 weeks of combined treatment with bleomycin arterial embolization, with platelet count and coagulation function returning to normal. After 4 weeks of bleomycin arterial embolization alone for 4 neonates, tumor volume was reduced by (52.0±3.4)%, and platelet count and coagulation function returned to normal. Blunt and sharp dissection was performed for 2 neonates. The tumor was removed completely during surgery in the 2 neonates, with no infection or recurrence after surgery, and platelet count and coagulation function returned to normal. The postoperative pathological examination showed Kaposiform hemangioendothelioma in 1 out of the 2 neonates.@*CONCLUSIONS@#KMS has characteristic clinical manifestations, histopathological features, and laboratory examination results. The KMS neonates who are not sensitive to glucocorticoids can achieve a good curative effect through arterial embolization and sirolimus treatment.

Síndrome de Kasabach- Merrit / Kasabach-Merritt syndrome

Introducción: El síndrome de Kasabach Merritt (SKM) es poco frecuente, tiene una alta mortalidad y se caracteriza por una lesión vascular de crecimiento rápido, asociado a coagulopatía de consumo y trombocitopenia. Materiales y métodos: revisión de literatura actual confrontando con artículos de revisiones de temas en búsqueda electrónica en bases de datos de RIMA, MEDLINE, PUBMED, MEDSCAPE, de 1940 a 2017. Resultados Se describe un caso clínico de un niño de 10 meses de edad con Síndrome de Kasabach Merrit sucedido en el Hospital Infantil Napoleón Franco Pareja (HINFP) Conclusiones El síndrome Kasabach-Merritt es una entidad poco frecuente, su diagnóstico inicia desde la sospecha clínica, que incluye al examen físico el hallazgo de un hemangioma acompañado de trombocitopenia y coagulopatía. El manejo no está pautado, se dispone de corticoide como terapia de inicio, interferón, antineoplásico, radioterapia o cirugía, y su pronóstico va a depender de la resolución pronta.

Introduction: The Kasabach-Merritt syndrome (SKM) is rare, has a high mortality and is characterized by a vascular lesion of rapid growth, associated with coagulopathy of consumption and thrombocytopenia. Materials and methods: review of current literature in comparison with articles of reviews of subjects in electronic search in databases of RIMA, MEDLINE, PUB-MED, MEDSCAPE, from 1940 to 2017. Results A clinical case of a 10-month-old child is described. of age with Kasabach Merrit syndrome occurred at the Napoleon Franco Franco Children's Hospital (HINFP) Conclusions The Kasabach-Merritt syndrome is a rare entity, its diagnosis starts from the clinical suspicion, which includes the physical examination the finding of a hemangioma accompanied by thrombocytopenia and coagulopathy. The management is not scheduled, corticoid is available as start therapy, interferon, antineoplastic, radiotherapy or surgery, and its prognosis will depend on the prompt resolution.