Original Article

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Blood Res 2016; 51(4):

Published online December 23, 2016

https://doi.org/10.5045/br.2016.51.4.256

© The Korean Society of Hematology

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, and Keon Hee Yoo1,3,4*

1Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

2Department of Pediatrics, Chung-Ang University Hospital, Seoul, Korea.

3Department of Medical Device Management and Research, SAIHST, Sungkyunkwan University, Seoul, Korea.

4Stem Cell & Regenerative Medicine Institute, Samsung Medical Center, Seoul, Korea.

Correspondence to : 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. hema2170@skku.edu

Received: May 20, 2016; Revised: August 22, 2016; Accepted: September 27, 2016

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

Article

Original Article

Blood Res 2016; 51(4): 256-260

Published online December 23, 2016 https://doi.org/10.5045/br.2016.51.4.256

Copyright © The Korean Society of Hematology.

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, and Keon Hee Yoo1,3,4*

1Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

2Department of Pediatrics, Chung-Ang University Hospital, Seoul, Korea.

3Department of Medical Device Management and Research, SAIHST, Sungkyunkwan University, Seoul, Korea.

4Stem Cell & Regenerative Medicine Institute, Samsung Medical Center, Seoul, Korea.

Correspondence to: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. hema2170@skku.edu

Received: May 20, 2016; Revised: August 22, 2016; Accepted: September 27, 2016

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

Fig 1.

Figure 1.

Probabilities of hematologic complete response (HCR) and clinical complete response (CCR). (A) The median time to achieve HCR was 157 days. The probability of achieving HCR at 1 year and 2.5 years was 77% and 88%, respectively. (B) The median time to achieve CCR was 332 days. The probability of achieving CCR at 1 year and 2.5 years was 54% and 86%, respectively.

Blood Research 2016; 51: 256-260https://doi.org/10.5045/br.2016.51.4.256

Table 1 . Treatment details of 13 patients with KMS..

Abbreviations: KMS, Kasabach-Merritt syndrome; IFNα, interferon-alpha; NA, not applicable..


Table 2 . Clinical characteristics and outcomes of 13 patients with KMS..

Abbreviations: KMS, Kasabach-Merritt syndrome; PLT, platelet count; Rt, right; Lt, left; IFNα, interferon-alpha; HCR, hematologic complete response; CCR, clinical complete response; NA, not achieved until last follow-up..


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