Original Article

Analysis on clinical characteristics and treatment for Kasabach-Merritt phenomenon in 36 children with hemangioma

  • Shihao HUANG ,
  • Xiaojun YUAN
Expand
  • Department of Pediatric Hematology/Oncology, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China

Received date: 2023-12-20

  Online published: 2024-11-08

Abstract

Objective To analyze the clinical characteristics of pediatric hemangioma patients with Kasabach-Merritt phenomenon (KMP), and sum up its treatment experience. Methods The clinical data of children with KMP diagnosed from April 1, 2006 to December 31, 2021 were retrospectively analyzed. Results A total of 36 children with hemangioma were accompanied by KMP, accounting for 0.6% of the children with hemangioma. Five patients (13.9%) showed typical clinical manifestations of KMP in the early stage of the disease; 26 patients (72.2%) had superficial tumors with small lesions; 4 patients (11.1%) had deep-seated tumors with relatively larger lesions; 1 patient (2.8%) presented with hematochezia as the initial symptom, and the tumor was not detected. Median follow-up time was 86 months. All patients manifested thrombocytopenia at the time of diagnosis and the median platelet counts were 24.5(11.8-43.5)×109/L, which increased to 168.0(101.8-314.5)×109/L after treatment. Twenty-four patients (66.7%) received combined therapy, all of them got complete remission (CR). Twelve patients (33.3%) received monotherapy, of them, five patients got CR, three got partial remission (PR), four showed no response. The overall CR rate was 80.6%, PR rate was 8.3%, and NR rate was 11.1%. The 7-year overall survival rate was 88.9% and the fatality rate was 11.1%. Conclusions The masking of symptom was the characteristics of KMP. Appropriate laboratory testing should be performed as soon as possible for patients with dysfunction of blood coagulation or rapidly progressive hemangiomas.

Cite this article

Shihao HUANG , Xiaojun YUAN . Analysis on clinical characteristics and treatment for Kasabach-Merritt phenomenon in 36 children with hemangioma[J]. Journal of Clinical Pediatrics, 2024 , 42(11) : 917 -921 . DOI: 10.12372/jcp.2024.23e1206

References

[1] Kasabach HH, Merritt KK. Capillary hemangioma with extensive purpura: report of a case[J]. Arch Pediatr Adolesc Med, 1940, 59: 1063-1070.
[2] Huo J, Chen S, Li J, et al. Retroperitoneal kaposiform hemangioendothelioma with kasabach-merritt phenomenon in children: a case report and review of the literature[J]. Front Pediatr, 2023, 11: 1138689.
[3] Maza-Morales M, Valdés-Loperena S, Durán-McKinster LC, et al. The use of mTOR inhibitors for the treatment of kaposiform hemangioendothelioma. A systematic review[J]. Pediatr Dermatol, 2023, 40(3): 440-445.
[4] Ji Y, Chen S, Zhou J, et al. Sirolimus plus prednisolone vs sirolimus monotherapy for kaposiform heman-gioendothelioma: a randomized clinical trial[J]. Blood, 2022, 139(11): 1619-1630.
[5] Zhou J, Li Y, Qiu T, et al. Long-term outcomes of sirolimus treatment for kaposiform hemangioendothelioma: Continuing successes and ongoing challenges[J]. Int J Cancer, 2023, 153(3): 600-608.
[6] Wang L, Li J, Wu C, et al. Case report: Experience of a rare case of rebound of the Kasabach-Merritt phenomenon during sirolimus treatment in kaposiform hemangioendothelioma[J]. Front Pediatr, 2022, 10: 949950.
[7] Yao W, Li KL, Qin ZP, et al. Standards of care for Kasabach-Merritt phenomenon in China[J]. World J Pediatr, 2021, 17(2): 123-130.
[8] 中国儿童原发性免疫性血小板减少症诊断与治疗指南改编工作组, 中华医学会儿科学分会血液学组, 中华儿科杂志编辑委员会. 中国儿童原发性免疫性血小板减少症诊断与治疗改编指南(2021版)[J]. 中华儿科杂志, 2021, 59(10): 810-819.
[9] Helligs? L, Mikkelsen TS, Hvas AM. Kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon in an infant girl[J]. Clin Case Rep, 2023, 11(9): e7859.
[10] Hu Y, Song D, Wu C, et al. Clinical and imaging features of Kaposiform hemangioendothelioma in infants[J]. Heliyon, 2023, 9(5): e15425.
[11] Zhao Y, Legan CE. Liver transplantation for giant hemangioma complicated by Kasabach-Merritt syndrome: a case report and literature review[J]. Am J Case Rep, 2022, 23: e936042.
[12] Zhang L, Liu L, Luo H, et al. Combination therapy for pediatric patients with Kasabach-Merritt phenomenon: a single-center retrospective study[J]. Medicine (Baltimore), 2022, 101(34): e30296.
[13] Yan Z, Ji C. Medical and interventional therapy of Kasabach-Merritt phenomenon associated with Kaposiform hemangioendothelioma: a case report[J]. Case Reports, 2023, 6(3): 130-133.
[14] Yin CG, Qi WW, Wang S, et al. Efficacy of transcatheter arterial chemoembolization combined with sirolimus for treating Kasabach-Merritt phenomenon in infants, a retrospective study[J]. Ann Med, 2023, 55(1):2196090.
Outlines

/