Peliosis hepatis


Peliosis hepatis

Description, Causes and Risk Factors:

Alternative Name: Peliosis hepatitis.

Abbreviation: PH.

ICD-10: K76.4

Peliosis hepatis a rare condition characterized by multiple small cystic blood-filled spaces from 1 mm to several centimeters in diameter in the hepatic parenchyma, resulting from the focal rupture of sinusoidal walls and may be associated with several diseases such as malignancy and acquired immune deficiency syndrome or medications such as anabolic steroids and oral contraceptives. Two varieties have been described: the phlebectatic type in which the blood-filled spaces are lined with endothelium and a consequence of an aneurysmal dilatation of the central vein, and the parenchymal type in which the blood spaces are not lined with endothelium and usually associated with hemorrhagic, parenchymal necrosis.

The morphogenesis of peliosis is controversial. It has been attributed to an increased sinusoidal pressure because of difficulties in blood out?ow from the liver, the disappearance of normal parenchyma by necrosis of liver cells, and sinusoid wall weakness. Since the disease is very rare, data about its natural history are scarce and clinical spectrum varies from asymptomatic cases to severe complications, such as hemoperitoneum.

A number of theories have been postulated to explain the etiology of peliosis hepatis, such as outflow obstruction at the sinusoidal level, hepatocellular necrosis, or direct lesions of the sinusoidal barrier. Peliosis hepatis is associated with a number of conditions including malignancies (particularly hepatocellular carcinoma), renal transplantation, hematologic disorders and infections such as pulmonary tuberculosis and human immunodeficiency virus infection. A bacterial (Rochalimaea henselae) causative agent in human immunodeficiency virus-related peliosis hepatis has recently been recognized, and regression can occur with appropriate antibiotic therapy. Bartonella henselae linked to cat and flea exposure. Peliosis in patients with long-term treatment with anabolic steroids, oral contraceptives, hormones, estrogen is also reported.

There have been few reports about the imaging findings of peliosis. CT might show multiple foci of decreased attenuation that might remain hypodense after intravenous contrast injection. There also might be areas of increased density. On MRI the lesions show increased signal intensity on T1-weighted images and variable signal intensity on T2-weighted images due to the presence of blood break down products.

Symptoms:

Clinical Signs May Include:

    Occurs at low CD4 counts; usually <100.

  • May be associated with immune reconstitution inflammatory syndrome (IRIS) and higher CD4 count during reconstitution.

  • Characterized by multiple, small, dilated blood-filled cavities in hepatic & splenic parenchyma.

  • Indolent course of fevers, nausea, abdominal pain, & malaise.

Diagnosis:

PH is difficult to recognize, and the diagnosis often is missed or delayed because its appearance on radiological imaging is suggestive of a neoplasm or multiple abscesses. An ultrasound scan may show hypoechoic areas involving the liver with intraperitoneal fluid collection and a normal Doppler signal. Occasionally, only subtle inhomogeneity is seen in the liver, which can also be seen in other diffuse liver parenchymal diseases. A contrast enhancement CT scan of the liver may show small lesions of a few millimeters to 1 to 4 cm in diameter. The lesions typically are hypodense in early arterial phase scans and enhanced in late venous phase. The CT appearance of peliosis hepatis can be difficult to differentiate from multiple abscesses, hemangiomatosis, and metastases. The diagnosis of peliosis hepatis on an angiography is made by visualizing multiple small accumulations of contrast material in the late arterial phase, which persists into the venous phase. This can be confused with irregular tumor vessels in focal nodular hyperplasia and adenomas. However, it is unclear if the angiography offers better diagnostic information than ultrasonography. In the current case, an emergency CT failed to show specific changes of peliosis hepatis other than that of hemoperitoneum caused by hepatorrhexis, and an angiogram was necessary to arrive at the diagnosis. A definitive diagnosis of peliosis hepatis was made from histological findings. A percutaneous needle biopsy can also be used to confirm the diagnosis. However, even when ultrasound-guided, the procedure has a high risk of a life-threatening hemorrhage. Consequently, a laparotomy appears to be the more appropriate procedure when tissue confirmation is needed because it permits assessment of the macroscopic appearance of peliosis, and a liver biopsy can be performed with adequate hemostasis.

Treatment:

There is no specific treatment for PH. Hepatic artery embolization or partial hepatectomy has been reported. Emergency liver transplantation is the ultimate treatment in cases of imminent liver failure. The natural course of peliosis hepatis is not well known. Reports have described outcomes ranging from spontaneous resolution to hepatic failure or fatal intraperitoneal hemorrhage. Some reports have described the resolution of peliosis hepatis after withdrawal of causative drugs or after treatment of the associated conditions. In other reports, patients presenting in fulminant hepatic failure eventually died. Fatal intraperitoneal hemorrhage has also been reported as another complication. Early removal of the known inciting agent may cause regression of this disease and prevent catastrophic complications.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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