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Successful treatment of a patient with Kasabach–Merritt syndrome and multiple giant hepatic hemangiomas

Authors
  • Liu, Yaqun1
  • Wu, Xin1
  • Ye, Lingying1
  • Xu, Huji1, 2, 3
  • 1 Department of Rheumatology and Immunology, Shanghai Changzheng Hospital, the Second Military Medical University, Shanghai, P.R. China
  • 2 nghua University, Beijing, P.R. China
  • 3 Peking-Tsinghua Center for Life Sciences, Tsinghua University, Beijing, P.R. China
Type
Published Article
Journal
The Journal of International Medical Research
Publisher
SAGE Publications
Publication Date
Jan 16, 2020
Volume
48
Issue
1
Identifiers
DOI: 10.1177/0300060519898358
PMID: 31948308
PMCID: PMC7113715
Source
PubMed Central
Keywords
License
Unknown

Abstract

Kasabach–Merritt syndrome (KMS) is a rare complication of hemangioma. KMS mostly occurs in the pediatric population with typical clinical manifestations, including thrombocytopenia, consumptive coagulation, and purpura. However, the pathogenesis of KMS is still unclear and the KMS therapy is controversial. We report here a case of KMS and multiple, giant, hepatic hemangiomas in a 34-year-old female patient who was successfully treated in our hospital. Glucocorticoid along with supportive treatments was administrated immediately to reverse fatal disseminated intravascular coagulation and acute hemolysis. After the acute phase, glucocorticoid was tapered slowly and sirolimus was added to treat the hemangiomas. In conclusion, the risk factors of gestation, interventional treatment, and autoimmune disturbance might contribute to the pathogenesis of KMS. Additionally, treatment with glucocorticoid and sirolimus is effective in KMS and multiple giant hepatic hemangiomas.

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