Peliosis hepatis

Peliosis hepatis
Classification and external resources
ICD-10	K76.4
MeSH	D010382

Peliosis hepatis is an uncommon vascular condition characterised by randomly distributed multiple blood-filled cavities throughout the liver. The 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]

1 Pathophysiology
2 Disease associations
3 Clinical features
4 Treatment
5 Other cystic conditions of liver
6 References

Pathophysiology

The pathogenesis of peliosis hepatis is unknown. There are several hypotheses, such as, it arises 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 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 consider both patterns 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^[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 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, 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

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

^ ^a ^b Sleisenger, Marvin (2006). Sleisenger and Fordtran's Gastrointestinal and Liver Disease. Philadelphia: W.B. Saunders Company. 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.

^ "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.

^ YANOFF M, RAWSON AJ (1964). "PELIOSIS HEPATIS. AN ANATOMIC STUDY WITH DEMONSTRATION OF TWO VARIETIES". Archives of pathology 77: 159–65.

^ 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.

^ 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.

^ 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.

^ 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.

^ 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.

^ Goldman, Lee (2003). Cecil Textbook of Medicine -- 2-Volume Set, Text with Continually Updated Online Reference. Philadelphia: W.B. Saunders Company.

Digestive system, Digestive disease, Gastroenterology (primarily K20–K93, 530–579)

Upper GI tract

Esophagus	Esophagitis Candidal Eosinophilic Herpetiform Rupture Boerhaave syndrome Mallory-Weiss syndrome UES Zenker's diverticulum LES Barrett's esophagus Esophageal motility disorder Nutcracker esophagus Achalasia Diffuse esophageal spasm Gastroesophageal reflux disease (GERD) Laryngopharyngeal reflux (LPR) Esophageal stricture Megaesophagus

Stomach	Gastritis Atrophic Ménétrier's disease Gastroenteritis Peptic (gastric) ulcer Cushing ulcer Dieulafoy's lesion Dyspepsia Pyloric stenosis Achlorhydria Gastroparesis Gastroptosis Portal hypertensive gastropathy Gastric antral vascular ectasia Gastric dumping syndrome Gastric volvulus

Lower GI tract:
Intestinal/
Enteropathy

Small intestine (Duodenum/Jejunum/Ileum)	Enteritis Duodenitis Jejunitis Ileitis Peptic (duodenal) ulcer Curling's ulcer Malabsorption: Coeliac Tropical sprue Blind loop syndrome Small bowel bacterial overgrowth syndrome Whipple's Short bowel syndrome Steatorrhea Milroy disease bile acid malabsorption

Large intestine (Appendix/Colon)	Appendicitis Colitis Pseudomembranous Ulcerative Ischemic Microscopic Collagenous Lymphocytic Functional colonic disease IBS Intestinal pseudoobstruction / Ogilvie syndrome Megacolon / Toxic megacolon Diverticulitis/Diverticulosis

Large and/or small	Enterocolitis Necrotizing IBD Crohn's disease Vascular: Abdominal angina Mesenteric ischemia Angiodysplasia Bowel obstruction: Ileus Intussusception Volvulus Fecal impaction Constipation Diarrhea Infectious Intestinal adhesions

Rectum	Proctitis Radiation proctitis Proctalgia fugax Rectal prolapse Anismus

Anal canal	Anal fissure/Anal fistula Anal abscess Anal dysplasia Pruritus ani

GI bleeding/BIS

Upper
- Hematemesis
- Melena
Lower
- Hematochezia

Accessory

Liver	Hepatitis Viral hepatitis Autoimmune hepatitis Alcoholic hepatitis Cirrhosis PBC Fatty liver NASH Vascular Budd-Chiari syndrome Hepatic veno-occlusive disease Portal hypertension Nutmeg liver Alcoholic liver disease Liver failure Hepatic encephalopathy Acute liver failure Liver abscess Pyogenic Amoebic Hepatorenal syndrome Peliosis hepatis Metabolic disorders Wilson's Disease Hemochromatosis

Gallbladder	Cholecystitis Gallstones/Cholecystolithiasis Cholesterolosis Rokitansky-Aschoff sinuses Postcholecystectomy syndrome Porcelain gallbladder

Bile duct/ Other biliary tree	Cholangitis PSC Secondary sclerosing cholangitis Ascending Cholestasis/Mirizzi's syndrome Biliary fistula Haemobilia Gallstones/Cholelithiasis Common bile duct Choledocholithiasis Biliary dyskinesia Sphincter of Oddi dysfunction

Pancreatic	Pancreatitis Acute Chronic Hereditary Pancreatic abscess Pancreatic pseudocyst Exocrine pancreatic insufficiency Pancreatic fistula

Abdominopelvic

Hernia	Diaphragmatic Congenital Hiatus Inguinal Indirect Direct Umbilical Femoral Obturator Spigelian Lumbar Petit's Grynfeltt-Lesshaft Undefined location Incisional Internal hernia

Peritoneal	Peritonitis Spontaneous bacterial peritonitis Hemoperitoneum Pneumoperitoneum

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Health science - Medicine - Cystic diseases

Respiratory system	Langerhans cell histiocytosis Lymphangiomyomatosis Cystic bronchiectasis

Skin	stratified squamous: follicular infundibulum Epidermoid cyst/Proliferating epidermoid cyst Milia Eruptive vellus hair cyst outer root sheath Trichilemmal cyst/Pilar cyst/Proliferating trichilemmal cyst/Malignant trichilemmal cyst sebaceous duct Steatocystoma multiplex/Steatocystoma simplex Keratocyst nonstratified squamous: Cutaneous ciliated cyst Hidrocystoma no epithelium: Pseudocyst of the auricle Mucocele other/ungrouped: Cutaneous columnar cyst Keratin implantation cyst Verrucous cyst Adenoid cystic carcinoma Breast cyst

Musculoskeletal system	Cystic hygroma

Digestive system	oral cavity: Cysts of the jaws Odontogenic cyst Radicular cyst Dentigerous cyst Odontogenic keratocyst Nasopalatine duct cyst liver: Polycystic liver disease Congenital hepatic fibrosis Peliosis hepatis bile duct: Biliary hamartomas Caroli disease Choledochal cysts Bile duct hamartoma

Nervous system	Cystic leukoencephalopathy

Genitourinary system	Polycystic kidney disease Autosomal dominant polycystic kidney Autosomal recessive polycystic kidney Medullary cystic kidney disease Nephronophthisis Congenital cystic dysplasia

Other conditions	Hydatid cyst Von Hippel-Lindau syndrome Tuberous sclerosis

Peliosis hepatis