Clinical features of Kasabach-Merritt syndrome: an analysis of 16 neonates.
- Author:
Ying-Hao WANG
1
;
Li-Ying DAI
1
;
Li-Li WANG
1
;
Jian ZHANG
1
;
Song WANG
;
Wei ZUO
Author Information
1. Department of Neonatology, Anhui Children's Hospital, Anhui Medical University, Hefei 230000, China.
- Publication Type:Journal Article
- MeSH:
Child;
Female;
Hemangioendothelioma;
Humans;
Infant, Newborn;
Kasabach-Merritt Syndrome/therapy*;
Male;
Neoplasm Recurrence, Local;
Retrospective Studies;
Sarcoma, Kaposi
- From:
Chinese Journal of Contemporary Pediatrics
2021;23(7):696-701
- CountryChina
- Language:Chinese
-
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.
