Journal List > Korean J Gastroenterol > v.67(4) > 1007501

Oak, Jun, Cho, Lee, Cho, Park, Joo, Kim, Rew, and Choi: Hepatic Hemangioma with KasabachMerritt Syndrome in an Adult Patient

Abstract

Hemangiomas are the most common benign tumors of the liver. They are generally asymptomatic, but giant hemangiomas can lead to abdominal discomfort, bleeding, or obstructive symptoms. KasabachMerritt syndrome is a rare but life-threatening complication of hemangioma, characterized by consumptive coagulopathy with large vascular tumors. More than 80% of KasabachMerritt syndrome cases occur within the first year of life. However, there are few reports of KasabachMerritt syndrome with giant hepatic hemangioma in adults and, as far as we know, no reports of KasabachMerritt syndrome with hepatic hemangioma treated with first line medical treatment only. The most important treatment for this syndrome is removal of the large vascular tumor. However, surgical treatment entails risk of bleeding, and the patient's condition can mitigate against surgery. We herein present a case of unresectable giant hepatic hemangioma with disseminated intravascular coagulopathy. The patient was a 60-year-old woman who complained of hematochezia, ecchymosis, and abdominal distension. She refused all surgical management and was therefore treated with systemic glucocorticoids and beta-blockers. After two weeks of steroid therapy, she responded partially to the treatment. Her laboratory findings and hematochezia improved. She was discharged on hospital day 33 and observed without signs of bleeding for three months.

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Fig. 1.
Physical examination at admission showed distended abdomen and extremity ecchymosis.
kjg-67-220f1.tif
Fig. 2.
Abdominal CT scan at presentation with several centripetal enhancing exophytic masses in both hepatic lobes (maximum size, 32.3×27.5×25.4 cm). (A) Cross section image. (B) Coronal section image. (C) Cross section image of abdominal CT 8 years prior (maximal size, 22.3 cm).
kjg-67-220f2.tif
Table 1.
Complete Blood Count and Coagulation Profile during Hospital Stay
  HD 1 a HD 3 a HD 5 a (steroid+1) HD 10 a (steroid+6) HD 16(steroid+12) HD 23(steroid+19) HD 29(steroid+25)
Hemoglobin (g/dL) 8.2 7.5 9.1 8.8 9.0 7.9 8.4
Platelet (/mm3) 32,000 29,000 36,000 39,000 40,000 54,000 92,000
PT (sec) 28.0 15.7 16.9 >100 18.0 14.2 13.1
aPTT (sec) >100 51.6 41.8 >100 39.8 37.4 40.0
Fibrinogen (mg/dL) <50 59.1 46.2 <50 <50 60 109.1
FDP (μ g/mL) 115.2 184.3     268.6 241.7 137.0

HD, hospital day; FDP, fibrinogen degradation products.

a Fresh frozen plasma, cryoprecipitate transfusion (HD 1-HD 10).

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