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CC BY-NC-ND 4.0 · AJP Rep 2020; 10(04): e390-e394
DOI: 10.1055/s-0040-1718901
DOI: 10.1055/s-0040-1718901
Case Report
Sirolimus for Kaposiform Hemangioendothelioma and Kasabach-Merritt Phenomenon in a Neonate
Abstract
We present a case of a neonate born with kaposiform hemangioendothelioma (KHE), complicated by Kasabach-Merritt phenomenon (KMP) and other serious conditions, who was successfully treated with sirolimus. In addition to complications from thrombocytopenia and fluid overload, during the course of therapy, our patient experienced supratherapeutic drug levels at the commonly accepted starting dose of sirolimus. Patients with KHE and KMP should be closely monitored for potential complications of both the initial disease and unexpected side effects of treatments.
Publication History
Received: 06 July 2020
Accepted: 21 August 2020
Article published online:
16 November 2020
© 2020. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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References
- 1 Zukerberg LR, Nickoloff BJ, Weiss SW. Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis. Am J Surg Pathol 1993; 17 (04) 321-328
- 2 Croteau SE, Liang MG, Kozakewich HP. et al. Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr 2013; 162 (01) 142-147
- 3 Merrow AC, Gupta A, Patel MN, Adams DM. 2014 revised classification of vascular lesions from the International Society for the Study of Vascular Anomalies: radiologic-pathologic update. Radiographics 2016; 36 (05) 1494-1516
- 4 Walsh MA, Carcao M, Pope E, Lee KJ. Kaposiform hemangioendothelioma presenting antenatally with a pericardial effusion. J Pediatr Hematol Oncol 2008; 30 (10) 761-763
- 5 el-Dessouky M, Azmy AF, Raine PA, Young DG. Kasabach-Merritt syndrome. J Pediatr Surg 1988; 23 (02) 109-111
- 6 Ryan C, Price V, John P. et al. Kasabach-Merritt phenomenon: a single centre experience. Eur J Haematol 2010; 84 (02) 97-104
- 7 Kasabach HH, Merritt KK. Capillary hemangioma with extensive purpura: report of a case. Am J Dis Child 1940; 59 (05) 1063-1070
- 8 Sarkar M, Mulliken JB, Kozakewich HP, Robertson RL, Burrows PE. Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma. Plast Reconstr Surg 1997; 100 (06) 1377-1386
- 9 Silvergleid AJ. Transfusion-associated circulatory overload (TACO). UpToDate. https://www.uptodate.com/contents/transfusion-associated-circulatory-overload-taco#:∼:text=S%20Tirnauer%2C%20MD-,INTRODUCTION,volume%20excess%20or%20circulatory%20overload . Accessed Aug 19, 2020
- 10 Politis C, Wiersum JC, Richardson C. et al. The International Haemovigilance Network Database for the Surveillance of Adverse Reactions and Events in Donors and Recipients of Blood Components: technical issues and results. Vox Sang 2016; 111 (04) 409-417
- 11 Shen WC, Liang PC, Wu JF, Hsu CT, Chen HS, Chen HL. Combined transarterial embolization and propranolol as effective treatment for hepatic hemangioma with Kasabach–Merritt syndrome and heart failure in a neonate. Pediatr Neonatol 2017; 58 (02) 191-193
- 12 Drolet BA, Trenor III CC, Brandão LR. et al. Consensus-derived practice standards plan for complicated Kaposiform hemangioendothelioma. J Pediatr 2013; 163 (01) 285-291
- 13 Anghelescu DL, De Armendi AJ, Thompson JW, Sillos EM, Pui CH, Sandlund JT. Vincristine-induced vocal cord paralysis in an infant. Paediatr Anaesth 2002; 12 (02) 168-170
- 14 Ahmed A, Williams D, Nicholson J. Vincristine-induced bilateral vocal cord paralysis in children. Pediatr Blood Cancer 2007; 48 (02) 248
- 15 Schmid I, Klenk AK, Sparber-Sauer M, Koscielniak E, Maxwell R, Häberle B. Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options. World J Pediatr 2018; 14 (04) 322-329
- 16 Adams DM, Trenor III CC, Hammill AM. et al. Efficacy and safety of sirolimus in the treatment of complicated vascular anomalies. Pediatrics 2016; 137 (02) e20153257
- 17 Vignot S, Faivre S, Aguirre D, Raymond E. mTOR-targeted therapy of cancer with rapamycin derivatives. Ann Oncol 2005; 16 (04) 525-537
- 18 Hemmings BA, Restuccia DF. PI3K-PKB/Akt pathway. Cold Spring Harb Perspect Biol 2012; 4 (09) a011189
- 19 Hammill AM, Wentzel M, Gupta A. et al. Sirolimus for the treatment of complicated vascular anomalies in children. Pediatr Blood Cancer 2011; 57 (06) 1018-1024
- 20 Marsh DJ, Trahair TN, Martin JL. et al. Rapamycin treatment for a child with germline PTEN mutation. Nat Clin Pract Oncol 2008; 5 (06) 357-361
- 21 Ji Y, Chen S, Xiang B. et al. Sirolimus for the treatment of progressive kaposiform hemangioendothelioma: a multicenter retrospective study. Int J Cancer 2017; 141 (04) 848-855
- 22 Russell TB, Rinker EK, Dillingham CS, Givner LB, McLean TW. Pneumocystis jirovecii pneumonia during sirolimus therapy for kaposiform hemangioendothelioma. Pediatrics 2018; 141 (Suppl. 05) S421-S424
- 23 Ying H, Qiao C, Yang X, Lin X. A case report of 2 sirolimus-related deaths among infants with Kaposiform Hemangioendotheliomas. Pediatrics 2018; 141 (Suppl. 05) S425-S429
- 24 Adams DM. A study to compare vincristine to sirolimus for treatment of high risk vascular tumors. NIH Study ID NCT02110069. Available at ClinicalTrials.gov: https://clinicaltrials.gov/ct2/show/record/NCT02110069 . Accessed January 5, 2020
- 25 Brattström C, Säwe J, Jansson B. et al. Pharmacokinetics and safety of single oral doses of sirolimus (rapamycin) in healthy male volunteers. Ther Drug Monit 2000; 22 (05) 537-544
- 26 Buhaescu I, Izzedine H, Covic A. Sirolimus--challenging current perspectives. Ther Drug Monit 2006; 28 (05) 577-584
- 27 Mizuno T, Emoto C, Fukuda T, Hammill AM, Adams DM, Vinks AA. Model-based precision dosing of sirolimus in pediatric patients with vascular anomalies. Eur J Pharm Sci 2017; 109S: S124-S131
- 28 Czechowicz JA, Long-Boyle JR, Rosbe KW, Mathes EF, Frieden IJ, Shimano KA. Sirolimus for management of complex vascular anomalies - a proposed dosing regimen for very young infants. Int J Pediatr Otorhinolaryngol 2018; 105: 48-51
- 29 Tribolet S, Hoyoux C, Boon LM. et al. A not so harmless mass: kaposiform hemangioendothelioma complicated by a Kasabach-Merritt phenomenon. Arch Pediatr 2019; 26 (06) 365-369
- 30 Wang H, Guo X, Duan Y, Zheng B, Gao Y. Sirolimus as initial therapy for kaposiform hemangioendothelioma and tufted angioma. Pediatr Dermatol 2018; 35 (05) 635-638
- 31 Schaefer BA, Wang D, Merrow AC, Dickie BH, Adams DM. Long-term outcome for kaposiform hemangioendothelioma: a report of two cases. Pediatr Blood Cancer 2017; 64 (02) 284-286
- 32 Zaidi SJ, Shaik S, Agrawal C, Cossor W. First intracardiac kaposiform hemangioendothelioma in an infant resolved with sirolimus: a case report. J Pediatr Hematol Oncol 2018; 40 (07) 536-540