Blood Res.  2016 Dec;51(4):256-260. 10.5045/br.2016.51.4.256.

Excellent outcome of medical treatment for Kasabach-Merritt syndrome: a single-center experience

• Kim JA
• Choi YB
• Yi ES
• Lee JW
• Sung KW
• Koo HH
• Yoo KH

• KMID: 2364316
• DOI: http://doi.org/10.5045/br.2016.51.4.256

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.

Keyword

Kasabach-Merritt syndrome; Treatment; Response; Prognosis

MeSH Terms

Cohort Studies
Female
Humans
Interferon-alpha
Kasabach-Merritt Syndrome*
Lost to Follow-Up
Male
Medical Records
Platelet Count
Prognosis
Propranolol
Vincristine
Interferon-alpha
Propranolol
Vincristine

Figure

• 

  Fig. 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.

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