Peliosis hepatis

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ICD10 = ICD10|K|76|4|k|70
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MeshID = D010382

Peliosis Hepatis is an uncommon vascular condition characterised by randomly distributed multiple blood-filled cavities throughout liver. Size of the cavities usually ranges between a few millimetres to 3 cm in diametercite book | last = Sleisenger | first = Marvin | title = Sleisenger and Fordtran's Gastrointestinal and Liver Disease | publisher = W.B. Saunders Company | location = Philadelphia | year = 2006 |isbn = 1416002456 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 anabolic steroids. It also occasionally affects spleen, lymph nodes, lungs, kidneys, adrenal glands, bone marrow and other parts of gastrointestinal tract. [cite journal |author=Ichijima K, Kobashi Y, Yamabe H, Fujii Y, Inoue Y |title=Peliosis hepatis. An unusual case involving multiple organs |journal=Acta Pathol. Jpn. |volume=30 |issue=1 |pages=109–20 |year=1980 |pmid=7361545] .

Peliosis hepatis is often erroneously written "peliosis hepati"ti"s", despite its not being one of the hepatitides. The correct term arises from the Greek "pelios", i.e. discoloured by extravasated blood, livid [cite web |url=http://www.perseus.tufts.edu/cgi-bin/ptext?doc=Perseus%3Atext%3A1999.04.0057%3Aentry%3D%2380479 |title=Henry George Liddell, Robert Scott, A Greek-English Lexicon |accessdate=2007-06-11 |format= |work=] , and the Latinized Genitive case (hepatis [cite web |url=http://www.perseus.tufts.edu/cgi-bin/ptext?doc=Perseus%3Atext%3A1999.04.0059%3Aentry%3D%2320439 |title=Charlton T. Lewis, Charles Short, A Latin Dictionary |accessdate=2007-07-02 |format= |work=] ) of the Greek "hepar", liver [cite web |url=http://www.perseus.tufts.edu/cgi-bin/ptext?doc=Perseus%3Atext%3A1999.04.0057%3Aentry%3D%2347443 |title=Henry George Liddell, Robert Scott, A Greek-English Lexicon |accessdate=2007-07-02 |format= |work=] .

Pathophysiology

The pathogenesis of peliosis hepatis is unknown. There are several hypotheses, such as, it arise from sinusoidal epithelial damage [cite journal |author=Gushiken FC |title=Peliosis hepatis after treatment with 2-chloro-3'-deoxyadenosine |journal=South. Med. J. |volume=93 |issue=6 |pages=625–6 |year=2000 |pmid=10881786] , increased sinusoidal pressure due to obstruction in blood outflow from the liver, or hepatocellular necrosis.

Two morphologic patterns of hepatic peliosis were described by Yanoff and Rawson [cite journal |author=YANOFF M, RAWSON AJ |title=PELIOSIS HEPATIS. AN ANATOMIC STUDY WITH DEMONSTRATION OF TWO VARIETIES |journal=Archives of pathology |volume=77 |issue= |pages=159–65 |year=1964 |pmid=14088761] . In the phlebectatic type, the blood-filled spaces are lined with endothelium and are associated with aneurysmal dilatation of the central vein; in the parenchymal type, the spaces have no endothelial lining and they usually are associated with haemorrhagic parenchymal necrosis. Some considers both pattern to be one process, initiated by focal necrosis of liver parenchyma observed in parenchymal type progressing into formation of fibrous wall and endothelial lining around haemorrhage of phebectatic type. Fibrosis, cirrhosis, regenerative nodules, and tumours may also be seen.

Disease associations

*Infections: HIV, Bacillary peliosis (caused by genus "Bartonella", bacteria responsible for cat-scratch disease which are identified histologically adjacent to the peliotic lesions [cite journal |author=Koehler JE, Sanchez MA, Garrido CS, Whitfeld MJ, Chen FM, Berger TG, Rodriguez-Barradas MC, LeBoit PE, Tappero JW |title=Molecular epidemiology of bartonella infections in patients with bacillary angiomatosis-peliosis |journal=N. Engl. J. Med. |volume=337 |issue=26 |pages=1876–83 |year=1997 |pmid=9407154 |doi=10.1056/NEJM199712253372603] ), "Staphylococcus aureus" [cite journal |author=Perkocha LA, Geaghan SM, Yen TS, Nishimura SL, Chan SP, Garcia-Kennedy R, Honda G, Stoloff AC, Klein HZ, Goldman RL |title=Clinical and pathological features of bacillary peliosis hepatis in association with human immunodeficiency virus infection |journal=N. Engl. J. Med. |volume=323 |issue=23 |pages=1581–6 |year=1990 |pmid=2233946]
*Chronic conditions: End stage renal failure, Kwashiorkor, tuberculosis and other chronic infections.
*Malignancy: Monoclonal gammopathies (multiple myeloma and Waldenstrom macroglobulinemia), Hodgkin disease, malignant histiocytosis, seminoma, hepatocellular adenoma and hepatocarcinoma, [cite journal |author=Haboubi NY, Ali HH, Whitwell HL, Ackrill P |title=Role of endothelial cell injury in the spectrum of azathioprine-induced liver disease after renal transplant: light microscopy and ultrastructural observations |journal=Am. J. Gastroenterol. |volume=83 |issue=3 |pages=256–61 |year=1988 |pmid=3278593]
* Renal 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 [cite journal |author=Izumi S, Nishiuchi M, Kameda Y, Nagano S, Fukunishi T, Kohro T, Shinji Y |title=Laparoscopic study of peliosis hepatis and nodular transformation of the liver before and after renal transplantation: natural history and aetiology in follow-up cases |journal=J. Hepatol. |volume=20 |issue=1 |pages=129–37 |year=1994 |pmid=8201214 |doi=10.1016/S0168-8278(05)80479-9] [cite journal |author=Cavalcanti R, Pol S, Carnot F, Campos H, Degott C, Driss F, Legendre C, Kreis H |title=Impact and evolution of peliosis hepatis in renal transplant recipients |journal=Transplantation |volume=58 |issue=3 |pages=315–6 |year=1994 |pmid=8053054] .
*Drugs and toxins: Corticosteroids, androgens, azathioprine, tamoxifen [cite book | last = Goldman | first = Lee | title = Cecil Textbook of Medicine -- 2-Volume Set, Text with Continually Updated Online Reference | publisher = W.B. Saunders Company | location = Philadelphia | year = 2003 | isbn = 0721645631 ]

Clinical features

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.

Treatment

Usually directed towards management of underlying cause. Withdrawal of azathioprine leads to remission in renal transplant, bacillary peliosis responds to antibiotics. In rare circumstances partial resection of liver or transplant may be required.

Other cystic conditions of liver

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

References