Peliosis hepatis is an uncommon Blood vessel condition characterised by multiple, randomly distributed, blood-filled cavities throughout the liver. The size of the cavities usually ranges between a few millimetres and 3 cm in diameter.[ Chapter 80] In the past, it was a mere histological curiosity occasionally found at autopsies, but has been increasingly recognised with wide-ranging conditions from AIDS to the use of . It also occasionally affects spleen, , , , , bone marrow, and other parts of gastrointestinal tract.
Peliosis hepatis is often erroneously written "peliosis hepati tis", despite its not being one of the hepatitis. The correct term arises from the Greek language pelios, i.e. discoloured by extravasated blood, livid,
Signs and symptoms
Often, peliosis hepatis causes no symptoms (asymptomatic). In other cases, it may be identified after blood tests show abnormalities in liver enzymes.
Less commonly, peliosis hepatis may cause abdominal pain, especially right upper quadrant pain, or jaundice.
Presentation
Disease associations
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Infections: HIV, bacillary peliosis (caused by genus Bartonella, bacteria responsible for cat-scratch disease which are identified histologically adjacent to the peliotic lesions
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Chronic conditions: End stage kidney failure, kwashiorkor, tuberculosis, and other chronic infections
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Malignancy: Monoclonal gammopathies (multiple myeloma and Waldenström's macroglobulinemia), Hodgkin disease, malignant histiocytosis, seminoma, hepatocellular adenoma, and hepatocarcinoma
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Kidney transplants: It can be found in up to 20% patients, can be related to azathioprine or cyclosporine use, and may be associated with increased risk of transplant rejection.
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Drugs and toxins: Corticosteroids, androgens, azathioprine, tamoxifen
Pathophysiology
The
pathogenesis of peliosis hepatis is unknown. Several
hypotheses are given, such as it arises from sinusoidal epithelial damage,
increased sinusoidal pressure due to obstruction in blood outflow from the liver, or hepatocellular
necrosis.
Peliosis hepatis is associated with infections, cancers, organ transplantation, blood disorders, autoimmune conditions, and certain medications.
Two morphologic patterns of hepatic peliosis were described by Yanoff and Rawson.
Diagnosis
The condition is typically asymptomatic and is discovered following evaluation of abnormal liver function test. However, when severe, it can manifest as
jaundice,
hepatomegaly,
liver failure, and
haemoperitoneum.
Other cystic conditions of liver
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Polycystic liver disease
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Solitary congenital cysts
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Congenital hepatic fibrosis
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Echinococcosis
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Von Meyenburg complexes
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Caroli disease (type V choledochal cyst)
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Type IV choledochal cysts
Treatment
Treatment is usually directed towards management of the underlying cause. Withdrawal of azathioprine leads to remission in kidney transplant; bacillary peliosis responds to antibiotics. In rare circumstances partial resection of liver or transplant may be required.
Epidemiology
Peliosis hepatis usually affects adults, though may occur in children.
Peliosis hepatis occurs equally in men and women.
History
Peliosis hepatis was first described in 1861.
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