Clinical features of Kasabach-Merritt syndrome: an analysis of 16 neonates / 中国当代儿科杂志
Chinese Journal of Contemporary Pediatrics
; (12): 696-701, 2021.
Article
在 Zh
| WPRIM
| ID: wpr-888468
Responsible library:
WPRO
ABSTRACT
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.
全文:
1
索引:
WPRIM
主要主题:
Sarcoma, Kaposi
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Retrospective Studies
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Kasabach-Merritt Syndrome
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Hemangioendothelioma
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Neoplasm Recurrence, Local
研究类型:
Observational_studies
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Prognostic_studies
限制:
Child
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Female
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Humans
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Male
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Newborn
语言:
Zh
期刊:
Chinese Journal of Contemporary Pediatrics
年:
2021
类型:
Article