Case Report: Kasabach–Merritt Phenomenon Associated with Hepatic Angiosarcoma
Key findings
- A 55-year-old previously healthy man presented to the emergency department with a one-month history of spontaneous hematomas and hematochezia
- Imaging revealed severe hepatomegaly (30 cm in largest dimension) and innumerable hepatic lesions almost completely replacing underlying parenchyma
- The patient was diagnosed with hepatic angiosarcoma and Kasabach–Merritt phenomenon on the basis of laboratory tests and liver biopsy with venous pressure measurements
- Romiplostim, enoxaparin and tranexamic acid led to moderate improvement of the coagulopathy, but the patient died 13 months after initial presentation
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Hepatic angiosarcoma, a rare malignancy of the liver, has a poor prognosis. Median survival time is only about five months, and it's estimated that only about 3% of patients live longer than two years.
An even rarer circumstance is that hepatic angiosarcoma can be complicated by Kasabach–Merritt phenomenon (KMP), a life-threatening intravascular coagulopathy. KMP arises because of platelet activation by abnormally proliferating endothelium and fibrinogen consumption.
In Gastroenterology, Eric M. Przybyszewski, MD, and Amiko M. Uchida, MD, both fellows in the Division of Gastroenterology at Massachusetts General Hospital, and Vikram Deshpande, MD, associate pathologist with the Mass General Gastrointestinal Pathology Unit in the Department of Pathology and the Mass General Cancer Center, report how they recognized and managed a patient with hepatic angiosarcoma and KMP.
Presentation
A 55-year-old previously healthy man presented to the emergency department with a one-month history of spontaneous hematomas and hematochezia. Two weeks prior, he had banding of newly developed internal hemorrhoids. Physical examination revealed a large lumbosacral hematoma, hepatomegaly and red blood per rectum.
Relevant laboratory test results were:
- Hemoglobin: 8.3 g/dL
- Platelet count: 97,000/mm3
- International normalized ratio: 1.7
- Activated partial thromboplastin time: 47.5 seconds
- Lactate dehydrogenase: 188 U/L
- Fibrinogen: 50 mg/dL
- D-dimer: >10,000 ng/mL
- Reticulocyte index: 0.39, consistent with hypoproliferation
Imaging of the abdomen and pelvis demonstrated normal spleen but severe hepatomegaly (30 cm in largest dimension). Innumerable hepatic lesions almost completely replaced the underlying parenchyma, raising concern for malignancy.
Diagnosis
Transjugular liver biopsy revealed venous pressure of 4 mmHg in the right atrium, 6 mmHg in the free hepatic vein and 8 mmHg in the wedged hepatic vein. The calculated hepatic venous pressure gradient was 2 mmHg.
Liver histology showed replacement of normal sinusoidal cells by atypical endothelial cell proliferation. The atypical cells were positive for the oncogenes ERG and p53, and a molecular diagnostic assay was positive for p53 mutation. Collectively, these results were consistent with a diagnosis of hepatic angiosarcoma. There was no fibrosis.
Management
The patient was started on romiplostim, enoxaparin and tranexamic acid for the management of coagulopathy. That regimen led to moderate improvement and chemotherapy with gemcitabine and docetaxel was initiated. The patient was later switched to doxorubicin and palliative pazopanib. Unfortunately, he died on hospice care 13 months after the initial presentation.
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