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=Portal vein thrombosis
__NOTOC__
{{Infobox Disease |
  Name          = Portal vein thrombosis |
  Image          = Pfortaderthrombose001.png |
  Caption        = Portal vein thrombosis in computertomography. |
  DiseasesDB    = |
  ICD10          = {{ICD10|I|81||i|80}} |
  ICD9          = {{ICD9|452}} |
  ICDO          = |
  OMIM          = |
  MeshID        = |
}}
{{Portal vein thrombosis}}
{{CMG}}
{{CMG}}
==Overview==
==Overview==
'''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>. 
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>.
== Pathophysiology ==
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"/>.
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.


'''Portal vein thrombosis''' is a form of [[venous thrombosis]] affecting the [[hepatic portal vein]], which can lead to [[portal hypertension]] and reduction in the blood supply to the [[liver]].
===Causes===


==Causes==
===Drug Causes===
Causes can include [[pancreatitis]], [[cirrhosis]], [[diverticulitis]], and [[cholangiocarcinoma]].  It is also a known [[Complication (medicine)|complication]] of [[splenectomy]].<ref>Ali Cadili, Chris de Gara, "Complications of Splenectomy", ''The American Journal of Medicine'', 2008, pp 371-375.</ref> Drugs such as [[Romiplostim]].


==Diagnosis==
* [[Fluoxymesterone]]
The diagnosis of portal vein thrombosis is usually made by [[ultrasound]], [[computed tomography]] with contrast or [[magnetic resonance imaging]]. [[D-dimer]] levels in the blood blood may be elevated as a result of fibrin degradation.
* [[Oxandrolone]]
* [[Nandrolone]]
* [[oxymetholone]]


==Treatment==
== Disease associations ==
<gallery>
*'''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>
Image:Kavernoese Transformation nach Pfortaderthrombose 001.png|Portal vein thrombosis on computed tomography (left) and cavernous transformation of the portal vein after 1 year (right)</gallery>
*'''Chronic conditions:'''  End stage [[renal failure]], [[Kwashiorkor]], [[tuberculosis]] and other chronic infections.
Treatments include [[anticoagulant]]s, [[Shunt (medical)|shunt]]s, [[Coronary artery bypass surgery|bypass surgery]], and [[Organ transplant|transplant]]s.
*'''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>
* '''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>.
*'''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>


==Related Chapters==
== Clinical features ==
* [[Pylephlebitis]]
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]].


==References==
== Treatment ==
=== Antimicrobial regimen ===
* Bacillary peliosis hepatitis (peliosis hepatis caused by ''Bartonella spp.'')<ref> {{cite book | last = Gilbert | first = David | title = The Sanford guide to antimicrobial therapy | publisher = Antimicrobial Therapy | location = Sperryville, Va | year = 2015 | isbn = 978-1930808843 }}</ref>
 
:*Preferred regimen (1): [[Clarithromycin]] 500 mg bid for 4 months
:*Preferred regimen (6): [[Erythromycin]] 500 mg PO qid for 4 months
:*Preferred regimen (2): [[Doxycycline]] 100 mg PO bid for 4 months
*Special Consideration
:*Severe disease
::* Preferred regimen: [[Doxycycline]] 100 mg PO/IV bid for 4 months {{and}} [[Rifampin]]  300 mg PO bid for 4 months
 
== 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 ==
{{Reflist|2}}
{{Reflist|2}}


==External links==
{{Commons category|Portal vein thrombosis}}
{{Vascular diseases}}


{{DEFAULTSORT:Portal Vein Thrombosis}}
{{Gastroenterology }}
{{Cystic diseases}}
[[Category:Gastroenterology]]
[[Category:Pathology]]
[[Category:Needs patient information]]
[[Category:Mature chapter]]
[[Category:Hepatology]]
[[Category:Hepatology]]
[[Category:Vascular diseases]]


{{WikiDoc Help Menu}}
{{WikiDoc Sources}}




Revision as of 16:22, 2 June 2016

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]


Overview

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 diameter[1]. 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.[2].

Peliosis hepatis is often erroneously written "peliosis hepatitis", despite its not being one of the hepatitides. The correct term arises from the Greek pelios, i.e. discoloured by extravasated blood, livid[3], and the Latinized Genitive case (hepatis[4]) of the Greek hepar, liver[5].

Pathophysiology

The pathogenesis of peliosis hepatis is unknown. There are several hypotheses, such as, it arise from sinusoidal epithelial damage[6], increased sinusoidal pressure due to obstruction in blood outflow from the liver, or hepatocellular necrosis[1].

Two morphologic patterns of hepatic peliosis were described by Yanoff and Rawson [7]. In the phlebectatic type, the blood-filled spaces are lined with endothelium and are associated with aneurismal 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.

Causes

Drug Causes

Disease associations

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

Antimicrobial regimen

  • Bacillary peliosis hepatitis (peliosis hepatis caused by Bartonella spp.)[14]
  • Preferred regimen (1): Clarithromycin 500 mg bid for 4 months
  • Preferred regimen (6): Erythromycin 500 mg PO qid for 4 months
  • Preferred regimen (2): Doxycycline 100 mg PO bid for 4 months
  • Special Consideration
  • Severe disease
  • Preferred regimen: Doxycycline 100 mg PO/IV bid for 4 months AND Rifampin 300 mg PO bid for 4 months

Other cystic conditions of liver

References

  1. ↑ 1.0 1.1 Sleisenger, Marvin (2006). Sleisenger and Fordtran's Gastrointestinal and Liver Disease. Philadelphia: W.B. Saunders Company. ISBN 1416002456. Chapter 80
  2. ↑ Ichijima K, Kobashi Y, Yamabe H, Fujii Y, Inoue Y (1980). "Peliosis hepatis. An unusual case involving multiple organs". Acta Pathol. Jpn. 30 (1): 109–20. PMID 7361545.
  3. ↑ "Henry George Liddell, Robert Scott, A Greek-English Lexicon". Retrieved 2007-06-11.
  4. ↑ "Charlton T. Lewis, Charles Short, A Latin Dictionary". Retrieved 2007-07-02.
  5. ↑ "Henry George Liddell, Robert Scott, A Greek-English Lexicon". Retrieved 2007-07-02.
  6. ↑ Gushiken FC (2000). "Peliosis hepatis after treatment with 2-chloro-3'-deoxyadenosine". South. Med. J. 93 (6): 625–6. PMID 10881786.
  7. ↑ YANOFF M, RAWSON AJ (1964). "PELIOSIS HEPATIS. AN ANATOMIC STUDY WITH DEMONSTRATION OF TWO VARIETIES". Archives of pathology. 77: 159–65. PMID 14088761.
  8. ↑ Koehler JE, Sanchez MA, Garrido CS, Whitfeld MJ, Chen FM, Berger TG, Rodriguez-Barradas MC, LeBoit PE, Tappero JW (1997). "Molecular epidemiology of bartonella infections in patients with bacillary angiomatosis-peliosis". N. Engl. J. Med. 337 (26): 1876–83. PMID 9407154.
  9. ↑ Perkocha LA, Geaghan SM, Yen TS, Nishimura SL, Chan SP, Garcia-Kennedy R, Honda G, Stoloff AC, Klein HZ, Goldman RL (1990). "Clinical and pathological features of bacillary peliosis hepatis in association with human immunodeficiency virus infection". N. Engl. J. Med. 323 (23): 1581–6. PMID 2233946.
  10. ↑ Haboubi NY, Ali HH, Whitwell HL, Ackrill P (1988). "Role of endothelial cell injury in the spectrum of azathioprine-induced liver disease after renal transplant: light microscopy and ultrastructural observations". Am. J. Gastroenterol. 83 (3): 256–61. PMID 3278593.
  11. ↑ Izumi S, Nishiuchi M, Kameda Y, Nagano S, Fukunishi T, Kohro T, Shinji Y (1994). "Laparoscopic study of peliosis hepatis and nodular transformation of the liver before and after renal transplantation: natural history and aetiology in follow-up cases". J. Hepatol. 20 (1): 129–37. PMID 8201214.
  12. ↑ Cavalcanti R, Pol S, Carnot F, Campos H, Degott C, Driss F, Legendre C, Kreis H (1994). "Impact and evolution of peliosis hepatis in renal transplant recipients". Transplantation. 58 (3): 315–6. PMID 8053054.
  13. ↑ Goldman, Lee (2003). Cecil Textbook of Medicine -- 2-Volume Set, Text with Continually Updated Online Reference. Philadelphia: W.B. Saunders Company. ISBN 0721645631.
  14. ↑ Gilbert, David (2015). The Sanford guide to antimicrobial therapy. Sperryville, Va: Antimicrobial Therapy. ISBN 978-1930808843.


Template:Gastroenterology Template:Cystic diseases


Template:WikiDoc Sources


Encephalitis Table

Reference list includes:[1][2]

Disease Similarities Differentials
Meningitis Classic triad of fever, nuchal rigidity, and altered mental status Photophobia, phonophobia, rash associated with meningococcemia, concomitant sinusitis or otitis, swelling of the fontanelle in infants (0-6 months)
Brain abscess Fever, headache, hemiparesis Varies depending on the location of the abscess; clinically, visual disturbance including papilledema, decreased sensation; on imaging, a lesion demonstrates both ring enhancement and central restricted diffusion
Demyelinating diseases Ataxia, lethargy Multiple sclerosis: clinically, nystagmus, internuclear ophthalmoplegia, Lhermitte's sign; on imaging, well-demarcated ovoid lesions with possible T1 hypointensities (“black holes”)

Acute disseminated encephalomyelitis: ; on imaging, diffuse or multi-lesion enhancement, with indistinct lesion borders

Substance abuse Tremor, headache, altered mental status Varies depending on type of substance: prior history, drug-seeking behavior, attention-seeking behavior, paranoia, sudden panic, anxiety, hallucinations
Electrolyte disturbance Fatigue, headache, nausea Varies depending on deficient ions; clinically, edema, constipation, hallucinations; on EKG, abnormalities in T wave, P wave, QRS complex; possible presentations include arrhythmia, dehydration, renal failure
Stroke Ataxia, aphasia, dizziness Varies depending on classification of stroke; presents with positional vertigo, high blood pressure, extremity weakness
Intracranial hemorrhage Headache, coma, dizziness Lobar hemorrhage, numbness, tingling, hypertension, hemorrhagic diathesis
Trauma Headache, altered mental status Amnesia, loss of consciousness, dizziness, concussion, contusion


References

  1. ↑ Eckstein C, Saidha S, Levy M (2012). "A differential diagnosis of central nervous system demyelination: beyond multiple sclerosis". J Neurol. 259 (5): 801–16. doi:10.1007/s00415-011-6240-5. PMID 21932127.
  2. ↑ De Kruijk JR, Twijnstra A, Leffers P (2001). "Diagnostic criteria and differential diagnosis of mild traumatic brain injury". Brain Inj. 15 (2): 99–106. doi:10.1080/026990501458335. PMID 11260760.
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