Giant liver hemangioma with adult Kasabach-Merritt syndrome: Case report and literature review

Medicine (Baltimore). 2017 Aug;96(31):e7688. doi: 10.1097/MD.0000000000007688.

Abstract

Rationale: Adult Kasabach-Merritt syndrome associated with giant liver hemangioma is rare; to date, most reports have been single-case reports, and no multi-case reports or literature reviews are available.

Diagnoses: We conducted a retrospective analysis of 5 cases of adult Kasabach-Merritt syndrome associated with giant liver hemangioma treated at our hospital between 2011 and 2016. All 5 patients had varying severities of leukopenia, anemia, thrombocytopenia, prolonged prothrombin time, and hypofibrinogenemia.

Interventions: All the patients underwent surgery: 2 patients had left hemihepatectomy; 1 had enucleation; 1 had a right hemihepatectomy; and 1 had a left trisectionectomy.

Outcomes: The 5 patients had an average operative time of 6.9 hours and an average blood loss of 3200 mL. One patient developed a biliary fistula (grade II) after the operation. There was no mortality among 5 patients. The white blood cell counts, hemoglobin, platelets, and prothrombin times of all 5 patients returned to normal after the operation. To date, a total of 11 cases of adult Kasabach-Merritt syndrome associated with giant liver hemangioma have been reported, of which 8 patients underwent surgery, and their platelets and coagulation returned to normal after the operation.

Lessons: Adult Kasabach-Merritt syndrome associated with giant liver hemangioma is uncommon, and surgical treatment is risky. However, resection of the tumor corrected the abnormalities in hematological and coagulative systems.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Female
  • Humans
  • Kasabach-Merritt Syndrome / blood
  • Kasabach-Merritt Syndrome / complications*
  • Kasabach-Merritt Syndrome / diagnostic imaging
  • Kasabach-Merritt Syndrome / surgery*
  • Liver / diagnostic imaging
  • Liver / surgery
  • Liver Neoplasms / blood
  • Liver Neoplasms / complications*
  • Liver Neoplasms / diagnostic imaging
  • Liver Neoplasms / surgery*
  • Male
  • Middle Aged