Blood Res 2016; 51(4): 256-260  https://doi.org/10.5045/br.2016.51.4.256
Excellent outcome of medical treatment for Kasabach-Merritt syndrome: a single-center experience
Jin Ah Kim1, Young Bae Choi2, Eun Sang Yi1, Ji Won Lee1, Ki Woong Sung1, Hong Hoe Koo1, Keon Hee Yoo1,3,4
Department of Pediatrics, 1Samsung Medical Center, Sungkyunkwan University School of Medicine, 2Chung-Ang University Hospital, 3Department of Medical Device Management and Research, SAIHST, Sungkyunkwan University, 4Stem Cell & Regenerative Medicine Institute, Samsung Medical Center, Seoul, Korea
Correspondence to: Keon Hee Yoo, M.D., Ph.D.
Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul 06351, Korea
E-mail: hema2170@skku.edu
Received: May 20, 2016; Revised: August 22, 2016; Accepted: September 27, 2016; Published online: December 31, 2016.
© The Korean Journal of Hematology. All rights reserved.

cc This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Background
Kasabach-Merritt syndrome (KMS) is a rare but life-threatening illness. The purpose of this study is to report our single-center experience with KMS.
Methods
We reviewed the medical records of 13 patients who were diagnosed with KMS between 1997 and 2012 at Samsung Medical Center. Treatment response was defined as follows: 1) hematologic complete response (HCR) – platelet count >130×109/L without transfusion; 2) clinical complete response (CCR) – complete tumor disappearance or small residual vascular tumor displaying lack of proliferation for at least 6 months after treatment discontinuation.
Results
Participants included 7 male and 6 female patients. The median initial hemoglobin levels and platelet counts were 9.7 g/dL (range, 6.6‒11.6 g/dL) and 11×109/L (range, 3‒38×109/L), respectively. Twelve patients received corticosteroid and interferon-alpha as initial treatment, and the remaining patient received propranolol instead of corticosteroid. Two patients with unsatisfactory response to the initial treatment received weekly vincristine. Successful discontinuation of medication was possible at a median of 301 days (range, 137‒579) in all patients except one who was lost to follow-up. The median times to achieve HCR and CCR were 157 days and 332 days, respectively. The probabilities of achieving HCR and CCR were 77% and 54% at 1 year, and 88% and 86% at 2.5 years, respectively.
Conclusion
The prognosis of KMS in our cohort was excellent. Our data suggest that individualized treatment adaptation according to response may be very important for the successful treatment of patients with KMS.
Keywords: Kasabach-Merritt syndrome, Treatment, Response, Prognosis


Current Issue

ba_link01

Indexed/Covered by