Peliosis hepatis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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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].
Historical Perspective
There is limited information about the historical perspective of Peliosis hepatis
Classification
There is no established system for the classification of Peliosis hepatis
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
Peliosis hepatis may be caused by drugs, toxins, chronic wasting disease, malignancy, and infection. Peliosis Hepatitis may develop in individuals with renal or cardiac transplantation, but in about 20-50% of patients, they are no comorbidities.
common causes include
- Corticosteriods
- Oral Contraceptives
- Tuberculosis
- Malignancy particularly Hepatocellular Carcinoma
- Diabetes mellitus
- Necrotizing vasculitis
- Infection in AIDS, bacillary peliosis caused by Bartonella henselae and Bartonella quintana)
- Hematologic disorders. [8]
Differentiating Peliosis hepatis from Other Diseases
Peliosis hepatis must be differentiated from Hepatic Adenoma, Hemangioma, Focal Nodular Hyperplasia, Hepatic Abscess, Hypervascular Metastases, and Hepatocellular Carcinoma.[9] Polycystic liver disease, Congenital hepatic fibrosis, Solitary congenital cysts, Hydatid cyst, Von Meyenburg complexes, Caroli disease (type V choledochal cyst).[10]
Epidemiology and Demographics
The prevalence of Peliosis hepatis is approximately 0.2 percent of patients with pulmonary tuberculosis and about 22 percent in patients following renal transplant. It is most commonly associated with renal transplants. [11]), Staphylococcus aureus[12]
- Chronic conditions: End stage renal failure, Kwashiorkor, tuberculosis and other chronic infections.
- Malignancy: Monoclonal gammopathies (multiple myeloma and Waldenstrom macroglobulinemia), Hodgkin's disease, malignant histiocytosis, seminoma, hepatocellular adenoma and hepatocarcinoma,[13]
- 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[14][15].
- Drugs and toxins: Corticosteroids, androgens, methyltestosterone, azathioprine, tamoxifen[16]
Prognosis
Depending on if the right diagnosis was made, the prognosis may vary. Peliosis hepatis should not be drained. If mistaken for hepatic abscess and drained, it can lead to life-threatening hemorrhage. However, the prognosis is generally regarded as excellent.[17]
Diagnosis
Diagnostic Criteria
Peliosis Hepatis is primarily diagnosed based on the clinical presentation like jaundice, hepatomegaly, and liver failure. There are no established criteria for the diagnosis of Peliosis Hepatis. It is usually asymptomatic.
History and Symptoms
The majority of patients with Peliosis Hepatis are asymptomatic. Sign and symptoms of Peliosis Hepatis may include jaundice, hepatomegaly, liver failure, fever, and abdominal pain, imaging is necessary to rule out other differential diagnoses.[18]
Physical Examination
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.
Laboratory Findings
There are no diagnostic laboratory findings associated with Peliosis Hepatis
Imaging Findings
Ultrasound appears non-specific, usually demonstrating an irregular hypoechoic region or mass, and on Computerized Tomography (CT scan) shows multiple hypoattenuating lesions of variable size. Central bleeding may lead to areas of hyperattenuating and even dystrophic calcification. Magnetic resonance imaging (MRI) may typically show hypointense, hyperintense, or enhancement, usually centrifugal (from the center outward). Angiography may show hypervascularity with multiple vascular nodules
Other Diagnostic Studies
Treatment
Medical Therapy
- Bacillary peliosis hepatitis (peliosis hepatis caused by Bartonella spp.)[19]
- 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
Surgery
Prevention
See Also
- 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
- ↑ 1.0 1.1 Sleisenger, Marvin (2006). Sleisenger and Fordtran's Gastrointestinal and Liver Disease. Philadelphia: W.B. Saunders Company. ISBN 1416002456. Chapter 80
- ↑ 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.
- ↑ "Henry George Liddell, Robert Scott, A Greek-English Lexicon". Retrieved 2007-06-11.
- ↑ "Charlton T. Lewis, Charles Short, A Latin Dictionary". Retrieved 2007-07-02.
- ↑ "Henry George Liddell, Robert Scott, A Greek-English Lexicon". Retrieved 2007-07-02.
- ↑ Gushiken FC (2000). "Peliosis hepatis after treatment with 2-chloro-3'-deoxyadenosine". South. Med. J. 93 (6): 625–6. PMID 10881786.
- ↑ YANOFF M, RAWSON AJ (1964). "PELIOSIS HEPATIS. AN ANATOMIC STUDY WITH DEMONSTRATION OF TWO VARIETIES". Archives of pathology. 77: 159–65. PMID 14088761.
- ↑ https://www.ajronline.org/doi/10.2214/AJR.05.0167#:~:text=The%20cause%20of%20peliosis%20hepatis,chronic%20wasting%20diseases%20(e.g.%2C%20tuberculosis
- ↑ https://www.ajronline.org/doi/10.2214/AJR.05.0167#:~:text=The%20cause%20of%20peliosis%20hepatis,chronic%20wasting%20diseases%20(e.g.%2C%20tuberculosis
- ↑ https://www.ncbi.nlm.nih.gov/books/NBK554470/
- ↑ https://www.ncbi.nlm.nih.gov/books/NBK554470/
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Goldman, Lee (2003). Cecil Textbook of Medicine -- 2-Volume Set, Text with Continually Updated Online Reference. Philadelphia: W.B. Saunders Company. ISBN 0721645631.
- ↑ Cohen GS, Ball DS, Boyd-Kranis R, Gembala RB, Wurzel J (1994). "Peliosis hepatis mimicking hepatic abscess: fatal outcome following percutaneous drainage". J Vasc Interv Radiol. 5 (4): 643–5. doi:10.1016/s1051-0443(94)71572-4. PMID 7949724.
- ↑ "StatPearls". 2020. PMID 32119357 Check
|pmid=value (help). - ↑ Gilbert, David (2015). The Sanford guide to antimicrobial therapy. Sperryville, Va: Antimicrobial Therapy. ISBN 978-1930808843.