Background
Cirrhosis of the liver is frequently the last stage before liver failure. It is characterized by loss of the normal architecture with fibrosis. Cirrhosis can be broadly classified into two categories based upon the histologic and clinical-pathogenesis.
Central-Based Fibrotic Lesions Alcoholic fibrosis and cirrhosis
Cardiac fibrosis
Venous outflow obstruction such as Budd-Chiari syndrome and veno-occlusive diseasePortal-Based Fibrotic Lesions Chronic hepatitis and cirrhosis
Biliary tract disease including pirmary biliary cirrhosis, sclerosing cholangitis, inflammatory changes associated with idiopathic inflammatory bowel disease, and duct obstructionThe pathologist must establish the diagnosis from tiny needle core biopsies. Histologic clues to the diagnosis include:
Fragmented specimen with rounded edges
Reticulin fibers at the edge of fragments
Portal tracts absent
Irregular pattern of central veins
Twin plates
Enlarged nuclei (large cell change)
Caution with capsule on wedge biopsies (subcapsular fibosis normally present).At times, the diagnosis may still be unclear. Pathologists may use the descriptive diagnoses such as probable or possible cirrhosis, or cannot exclude cirrhosis. If there is good clinical documentation of the duration of the cirrhosis of progression of the fibrosis, the term early cirrhosis has sometimes been used. If there is extensive fibrosis with minimal residual normal liver cells, the term end-stage cirrhosis has been used.
OUTLINE
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES DIABETIC HEPATOSCLEROSIS
- Diabetic hepatosclerosis: diabetic microangiopathy of the liver.
Harrison SA, Brunt EM, Goodman ZD, Di Bisceglie AM.
Liver Center, Department of Medicine, Saint Louis University School of Medicine, St Louis, MO 63110, USA.
Arch Pathol Lab Med. 2006 Jan;130(1):27-32. Abstract quote
CONTEXT: Liver disease associated with diabetes mellitus is common and usually takes the form of simple steatosis or nonalcoholic steatohepatitis. After observing a noncirrhotic form of hepatic sinusoidal fibrosis in patients with long-standing diabetes mellitus who underwent liver biopsy, we set about to characterize this novel entity.
DESIGN AND SETTING: Cases with the hallmark histologic findings were gathered at Saint Louis University School of Medicine and the Armed Forces Institute of Pathology.
PATIENTS: The clinical records were examined in a systematic fashion. Results of light microscopy and prepared immunohistochemical stains were reviewed.
MAIN OUTCOME MEASURES: Clinical findings of patients with the histologic, detailed light microscopic, and immunohistochemical findings on biopsies.
RESULTS: Twelve patients were identified from biopsy findings; all had a history of long-standing diabetes mellitus and a noncirrhotic form of hepatic sinusoidal fibrosis not associated with nonalcoholic steatohepatitis. Most of these patients had a body mass index less than 25 kg/m2 and had substantial evidence of microvascular complications, including retinopathy, nephropathy, and peripheral and autonomic neuropathy. Alkaline phosphatase elevation was common. Liver biopsy specimens showed extensive dense perisinusoidal fibrosis, and immunostaining revealed basement membrane components in a perisinusoidal distribution. Features of nonalcoholic steatohepatitis were not present in the biopsy specimens.
CONCLUSIONS: We propose the term diabetic hepatosclerosis for this entity and suggest that it represents a form of diabetic microangiopathy affecting the liver. Further studies are needed to precisely characterize diabetic hepatosclerosis and to understand mechanisms of pathogenesis and the clinical significanceIDIOPATHIC PORTAL HYPERTENSION
Liver pathology of idiopathic portal hypertension. Comparison with non-cirrhotic portal fibrosis of India. The Japan idiopathic portal hypertension study.Okuda K, Nakashima T, Okudaira M, Kage M, Aida Y, Omata M, Sugiura M, Kameda H, Inokuchi K, Bhusnurmath SR, Aikat BA.
Liver 1982 Sep;2(3):176-92 Abstract quote Morphological changes of the liver were studied in 24 autopsy cases of noncirrhotic portal hypertension of unknown etiology (idiopathic portal hypertension, IPH), and in 123 surgical biopsies from such patients.
For comparison, 15 whole-cut liver slices from autopsy cases of noncirrhotic portal fibrosis (NCPF) from India were also studied. Liver pathology was very similar in IPH and NCPF, characterized by phlebosclerotic changes and perivascular fibrosis of the portal vein system, and parenchymal atrophy perhaps secondary to portal circulatory insufficiency. The distribution of lesions was uneven, and despite marked fibrosis and occasional surface nodularity, there was no diffuse pseudonodule formation in the parenchyma. Surgical specimens showed similar changes except for more frequent portal cellular infiltrates, but the changes seen in one biopsy specimen were limited and not always diagnostic.
It seems that IPH of Japan and NCPF of India are the same disease, and perhaps hepatoportal sclerosis elsewhere is also the same disease.
Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature.Dhiman RK, Chawla Y, Vasishta RK, Kakkar N, Dilawari JB, Trehan MS, Puri P, Mitra SK, Suri S.
Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
J Gastroenterol Hepatol 2002 Jan;17(1):6-16 Abstract quote BACKGROUND: Non-cirrhotic portal fibrosis (NCPF), the equivalent of idiopathic portal hypertension in Japan and hepatoportal sclerosis in the United States of America, is a common cause of portal hypertension in India. The clinical features, portographic and histological findings, and management of 151 patients with non-cirrhotic portal fibrosis are presented.
METHODS: The disease is diagnosed by the presence of unequivocal evidence of portal hypertension in the definite absence of liver cirrhosis and extrahepatic portal vein obstruction (EHPVO). Retrospective analysis of records of 151 patients with NCPF was analyzed for the clinical presentation, physical findings, laboratory tests, radiological and histological findings, and for the outcome of treatment.
RESULTS: The disease is characterized by massive splenomegaly with anemia, preserved liver function and benign prognosis in a majority of patients. Splenoportovenography (SPV) showed massive dilatation of the portal and splenic veins, and the presence of collaterals. Twenty-four (15.9%) patients showed evidence of natural/spontaneous shunts (splenorenal 15, umbilical nine) on SPV; these patients had a lower incidence of variceal bleeding. Liver histology demonstrated maintained lobular architecture, portal fibrosis of variable degree, sclerosis and obliteration of small-sized portal vein radicles, and subcapsular scarring with the collapse of the underlying parenchyma. Piecemeal or hepatocytic necrosis was absent in all histology specimens. Three patients showed nodular transformation along with abnormal liver functions, and may represent late manifestation of NCPF where features are similar to those seen in patients with incomplete septal cirrhosis. In the initial part of the study, surgery (side-to-side lieno-renal shunt) was the preferred modality of treatment, however, endoscopic sclerotherapy or variceal ligation has now become the preferred first line of management of variceal bleeding.
CONCLUSIONS: The epidemiological and clinical features of NCPF have more similarity to IPH than has previously been documented. The development of spontaneous shunts tends to protect these patients from variceal bleeding.
Mod Pathol 2000;13:679-704.
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Last Updated January 20, 2006
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