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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
- Infections: HIV, Bacillary peliosis (caused by genus Bartonella, bacteria responsible for cat-scratch disease which are identified histologically adjacent to the peliotic lesions[8]), Staphylococcus aureus[9]
- 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,[10]
- 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[11][12].
- Drugs and toxins: Corticosteroids, androgens, methyltestosterone, azathioprine, tamoxifen[13]
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
- 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.
- ↑ 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.
- ↑ 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.
- ↑ Gilbert, David (2015). The Sanford guide to antimicrobial therapy. Sperryville, Va: Antimicrobial Therapy. ISBN 978-1930808843.
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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
- ↑ 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.
- ↑ 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.