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| {{Infobox_Disease
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| | {{Peliosis hepatis}} |
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| | ICD10 = {{ICD10|K|76|4|k|70}}
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| {{CMG}}
| | ==[[Peliosis hepatis overview|Overview]]== |
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| | ==[[Peliosis hepatis historical perspective|Historical Perspective]]== |
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| | ==[[Peliosis hepatis classification|Classification]]== |
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| ==Overview== | | ==[[Peliosis hepatis pathophysiology|Pathophysiology]]== |
| '''Peliosis Hepatis''' is an uncommon [[Blood vessel|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 diameter<ref name="marvin">{{cite 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 </ref>. 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 steroid]]s. It also occasionally affects [[spleen]], [[lymph nodes]], [[lungs]], [[kidneys]], [[adrenal glands]], [[bone marrow]] and other parts of [[gastrointestinal tract]].<ref>{{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}}</ref>.
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| Peliosis hepatis is often erroneously written "peliosis hepati''ti''s", despite its not being one of the [[hepatitis|hepatitides]]. The correct term arises from the Greek ''pelios'', i.e. discoloured by extravasated blood, livid<ref>{{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=}}</ref>, and the Latinized Genitive case (hepatis<ref>{{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=}}</ref>) of the Greek ''hepar'', liver<ref>{{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=}}</ref>. | | ==[[Peliosis hepatis causes|Causes]]== |
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| == Pathophysiology == | | ==[[Peliosis hepatis differential diagnosis|Differentiating Peliosis hepatis from Other Diseases]]== |
| The [[pathogenesis]] of peliosis hepatis is unknown. There are several [[Hypothesis|hypotheses]], such as, it arise from [[sinusoidal]] epithelial damage<ref>{{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}}</ref>, increased sinusoidal pressure due to obstruction in blood outflow from the liver, or hepatocellular [[necrosis]]<ref name="marvin"/>.
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| Two [[morphology (biology)|morphologic]] patterns of hepatic peliosis were described by Yanoff and Rawson <ref>{{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}}</ref>. In the '''phlebectatic''' type, the blood-filled spaces are lined with [[endothelium]] and are associated with [[aneurysm|aneurism]]al dilatation of the central vein; in the '''parenchymal''' type, the spaces have no endothelial lining and they usually are associated with [[haemorrhagic]] [[parenchyma]]l [[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 [[tumor|tumours]] may also be seen.
| | ==[[Peliosis hepatis epidemiology and demographics|Epidemiology and Demographics]]== |
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| ===Causes=== | | ==[[Peliosis hepatis risk factors|Risk Factors]]== |
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| ===Drug Causes=== | | ==[[Peliosis hepatis screening|Screening]]== |
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| * [[Fluoxymesterone]]
| | ==[[Peliosis hepatis natural history, complications and prognosis|Natural History, Complications, and Prognosis]]== |
| * [[Oxandrolone]]
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| * [[Nandrolone]]
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| * [[oxymetholone]]
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| == Disease associations == | | ==Diagnosis== |
| *'''Infections:''' [[HIV]], Bacillary peliosis (caused by genus ''[[Bartonella]]'', bacteria responsible for [[cat-scratch disease]] which are identified histologically adjacent to the peliotic lesions<ref>{{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}}</ref>), ''[[Staphylococcus aureus]]''<ref>{{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}}</ref>
| | [[Peliosis hepatis history and symptoms|History and Symptoms]] | [[Peliosis hepatis physical examination|Physical Examination]] | [[Peliosis hepatis laboratory findings|Laboratory Findings]] | [[Peliosis hepatis electrocardiogram|Electrocardiogram]] | [[Peliosis hepatis chest x ray|Chest X Ray]] | [[Peliosis hepatis CT|CT]] | [[Peliosis hepatis MRI|MRI]] | [[Peliosis hepatis echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Peliosis hepatis other imaging findings|Other Imaging Findings]] | [[Peliosis hepatis other diagnostic studies|Other Diagnostic Studies]] |
| *'''Chronic conditions:''' End stage [[renal failure]], [[Kwashiorkor]], [[tuberculosis]] and other chronic infections.
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| *'''Malignancy:''' [[Monoclonal gammopathy|Monoclonal gammopathies]] ([[multiple myeloma]] and [[Waldenstrom macroglobulinemia]]), [[Hodgkin's disease]], [[malignant histiocytosis]], [[seminoma]], [[hepatocellular adenoma]] and [[Hepatocellular carcinoma|hepatocarcinoma]],<ref>{{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}}</ref>
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| * '''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]]<ref>{{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}}</ref><ref>{{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}}</ref>.
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| *'''Drugs and toxins:''' [[Corticosteroids]], [[androgens]], [[methyltestosterone]], [[azathioprine]], [[tamoxifen]]<ref>{{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 }}</ref>
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| == Clinical features == | | ==Treatment== |
| 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 [[Hemoperitoneum|haemoperitoneum]].
| | [[Peliosis hepatis medical therapy|Medical Therapy]] | [[Peliosis hepatis surgery|Surgery]] | [[Peliosis hepatis primary prevention|Primary Prevention]] | [[Peliosis hepatis secondary prevention|Secondary Prevention]] | [[Peliosis hepatis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Peliosis hepatis future or investigational therapies|Future or Investigational Therapies]] |
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| == Treatment == | | ==Case Studies== |
| Usually directed towards management of underlying cause. Withdrawal of azathioprine leads to remission in [[Kidney transplantation|renal transplant]], bacillary peliosis responds to antibiotics. In rare circumstances partial resection of liver or [[Liver transplantation|transplant]] may be required.
| | [[Peliosis hepatis case study one|Case #1]] |
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| If caused by Bartonella , the treatment regimen includes [[Erythromycin]] 500 mg PO qid {{or}} [[Doxycycline]] 100 mg PO bid for 4 months.<ref>{{cite book | last = Bartlett | first = John | title = Johns Hopkins ABX guide : diagnosis and treatment of infectious diseases | publisher = Jones and Bartlett Learning | location = Burlington, MA | year = 2012 | isbn = 978-1449625580 }}</ref>
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| == 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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| *[[Hydatid cyst]]
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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]]
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| == References ==
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| {{Reflist|2}}
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| {{Gastroenterology }}
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| {{Cystic diseases}}
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| [[de:Peliosis hepatis]]
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| [[Category:Hepatology]] | | [[Category:Hepatology]] |
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