Overview

Peliosis hepatis (PH) was first described in 1861. PH is a very rare disease, more common in adults, and when it affects the liver, it comes to the surgeon’s attention only in an extremely urgent situation after the lesion’s rupture with the resulting hemoperitoneum. Peliosis hepatis (PH) is a disease characterized by multiple and small, blood-filled cysts within the parenchymatous organs. The cysts of peliosis hepatis often lack a cell lining and are surrounded by hepatocytes. Some have an endothelial cell lining, accompanied by dilated hepatic sinusoids. The cause is probably damage to the sinusoidal lining cells. Peliosis hepatis is associated with use of hormones (eg, anabolic steroids, oral contraceptives, glucocorticoids), tamoxifen, vinyl chloride, vitamin A and, particularly in kidney transplant recipients, azathioprine. Peliosis hepatis usually affects adults, though may occur in children. Peliosis hepatis occurs equally in men and women.

Causes

• Infections: HIV, bacillary peliosis (caused by genus Bartonella, bacteria responsible for cat-scratch disease which are identified histologically adjacent to the peliotic lesions), Staphylococcus aureus
• Chronic conditions: End stage kidney failure, kwashiorkor, tuberculosis, and other chronic infections
• Malignancy: Monoclonal gammopathies (multiple myeloma and Waldenström’s macroglobulinemia), Hodgkin disease, malignant histiocytosis, seminoma, hepatocellular adenoma, and hepatocarcinoma
• 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.
• Drugs and toxins: Corticosteroids, androgens, azathioprine, tamoxifen

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. Peliosis hepatis may cause abdominal pain, especially right upper quadrant pain, or jaundice.

Diagnosis

Liver function test is enough to diagnose the disease.

• Polycystic liver disease
• Solitary congenital cysts
• Congenital hepatic fibrosis
• Hydatid cyst
• Von Meyenburg complexes
• Caroli disease (type V choledochal cyst)
• 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.

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