Imaging of nodular regenerative hyperplasia of the liver. Study of 9 cases

Radiologic patterns of the liver in nodular regenerative hyperplasia are poorly known. The authors describe the features of the liver observed during ultrasonography, angiography, computed tomography and magnetic resonance imaging in 9 patients with nodular regenerative hyperplasia. Generally the liver appeared normal, occasionally with signs of portal hypertension. However, a pseudotumoral pattern was observed in 2 patients: they had well limited hypoechoic lesions on ultrasonography, and in one patient angiography showed hypervascularity.     

Nodular regenerative hyperplasia of the liver and antiphospholipid antibodies: report of two cases and review of the literature

Nodular regenerative hyperplasia of the liver (NRHL) is a rare disorder characterized by diffuse micronodular transformation of the hepatic parenchyma without fibrous septa between the nodules. This condition appears to be associated in many occasions with systemic autoimmune diseases. We describe two new patients with NRHL in whom antiphospholipid antibodies (aPL) were detected in their sera and review the few similar cases reported previously. We also discuss the possible relationship between aPL and NRHL and suggest that these antibodies may play a role in the pathogenesis of some cases of NRHL, specially those with an associated antiphospholipid syndrome.     

Micronodular transformation (nodular regenerative hyperplasia) of the liver: a report of 64 cases among 2,500 autopsies and a new classification of benign hepatocellular nodules

Nodular regenerative hyperplasia is defined by hepatocellular nodules distributed throughout the liver in the absence of fibrous septa between the nodules. Most reports have been single cases so that the prevalence and clinical significance of nodular regenerative hyperplasia is uncertain. In this study, the hepatic histology of 2,500 consecutive autopsies was reviewed. A spectrum of nodular transformation was found with nodular regenerative hyperplasia present in 2.6% of autopsy livers and qualitatively similar but lesser degrees of nodular transformation in a further 10.2%. Nodular transformation was also seen in 47% of livers with cirrhosis and 69% with incomplete cirrhosis. Obliteration of many small portal veins was seen in all cases with nodular regenerative hyperplasia, but only 4.7% of these had evidence of portal hypertension. The prevalence of various clinical states was compared in nodular regenerative hyperplasia and in controls. The results confirm, extend and quantify the spectrum of associated diseases. Nodular regenerative hyperplasia occurs in 5.6% of individuals over age 80 and with increased frequency in patients with systemic arteritis, polymyalgia rheumatica, massive tumor infiltration and mineral oil deposition. Nodular regenerative hyperplasia appears to be the hepatic analogue of arterial and arteriolar nephrosclerosis. A new classification of nodular transformation is proposed that encompasses the spectrum of lesions described here and the previously defined entities of focal nodular hyperplasia, partial nodular transformation and "cirrhosis telangiectasia hepatis." The major conclusion is that nodular regenerative hyperplasia is a secondary and nonspecific tissue adaptation to heterogeneous distribution of blood flow and does not represent a specific entity.     

Diffuse nodular regenerative hyperplasia of the liver (DNRH). A clinicopathologic study of 24 cases

The authors report 24 cases of diffuse nodular regenerative hyperplasia of the liver (DNRH) seen in a General Hospital during the last 9 years (prevalence: 3'1/100,000, incidence: 0'34/100,000). DNRH was diagnosed in 0.52% of the liver biopsies and 0.72 of the autopsies. These results suggest that DNHR is probably more frequent than suspected, and 1 DNRH was seen for each 39 biopsied cases of liver cirrhosis. Fourteen patients did not have hepatic symptoms. Portal hypertension was present in 9 cases. The biochemical disturbance most frequently found was a moderate elevation of GGT and APh, associated with slight elevation of SGOT, SGPT and bilirubin levels. Normal liver function tests could be seen (3 cases). Previous exposure to potentially hepatotoxic drugs or chemicals was discovered in 15 cases (62.5%). Diseases associated were circulatory disturbances (6 cases), autoimmune disease (5 cases), hemopathies (5 cases), and visceral carcinomas (4 cases). Two patients were recipients of renal transplant. Nodules distributed through the whole liver tissue were found in 16 cases, while 8 patients showed areas of normal parenchyma in their livers. Impairment of small hepatic vessels was detected in 16 cases. Some uneven cytologic findings were discovered: clusters of small basophilic cells (4 cases), large clear cells (8 cases), and dysplastic hepatocytes (10 cases), which suggests that DNRH could be a preneoplastic condition.     

Systemic lupus erythematosus and nodular regenerative hyperplasia of the liver

A patient with systemic lupus erythematosus and nodular regenerative hyperplasia of the liver is presented.     

对肝结节状再生性增生的再认识—附18例临床分析

目的通过对18例肝结节状再生性增生(nodular regenerative hyperplasia of liver,NRH)病例进行总结分析,以提高临床医师对此类疾病的认识。方法对北京协和医院1983年1月~2008年12月住院的18例NRH患者的临床表现、实验室检查、影像学检查及病理学检查等多项资料进行总结和分析。结果NRH临床上可表现为肝脏弥漫性病变和占位性病变,但以前者多见。门脉高压症是NRH常见的临床表现,以脾大、脾亢较为突出,肝功能仅轻度异常。NRH常合并自身免疫或胶原血管性疾病,此类患者常伴有多种自身抗体、ESR增快、Ig增高、γ%增高等异常。病理学检查肝脏内弥漫分布小的无纤维分隔的再生结节,其门静脉分支有不同程度的狭窄或闭塞。结论对临床上难以解释或不明原因的门脉高压症患者,尤其是合并自身免疫性疾病的患者,要警惕NRH的可能性。     

Budd-Chiari syndrome associated with nodular regenerative hyperplasia of the liver

The Budd-Chiari syndrome due to membranous obstruction of the hepatic blood outflow tract is a rare condition in western countries, and its association with nodular regenerative hyperplasia of the liver has never been described. We present the case of a 34-year-old woman with membranous obstruction of hepatic veins and nodular regenerative hyperplasia of the liver. Although webs have been difficult to demonstrate by sonography, we were able to image a structure in the hepatic vein near the junction with the inferior vena cava, suggesting a membranous nature.     

Encephalopathy following portal shunting in nodular regenerative hyperplasia of the liver

Two patients with nodular hyperplasia of the liver developed a chronic disabling encephalopathy after an interposition mesocaval shunt. Both had a low total hepatic blood flow-rate postoperatively. Encephalopathy disappeared following surgical occlusion of the shunt. These observations emphasize the risk of postshunt encephalopathy in patients with non-cirrhotic intrahepatic portal hypertension.     

Nodular regenerative hyperplasia of the liver in rheumatic diseases: report of seven cases and review of the literature

Nodular regenerative hyperplasia (NRH) of the liver is an uncommon pathologic finding associated, in most cases, with rheumatic and hematologic diseases. Although its pathogenesis remains unclear, NRH probably results from liver regeneration to maintain its functional capacity after ischemia-induced injury. An intrahepatic microvascular occlusive mechanism has been considered most likely pathogenetically. NRH may lead to portal hypertension. Thus, the diagnosis of Felty's syndrome must be considered with caution in patients with rheumatoid arthritis (RA) and NRH of the liver. We report seven additional cases of NRH in patients with rheumatic disorders and review the literature to determine the patterns of clinical presentation and natural history of this condition. We also report four patients (three systemic lupus erythematosus [SLE] and one primary antiphospholipid syndrome [PAPS]) in whom antiphospholipid antibodies may have played a role in the genesis of NRH.     

Nodular regenerative hyperplasia of the liver. Study of 15 cases and review of the literature

The authors report 15 cases of nodular regenerative hyperplasia (NRH) of the liver observed in 10 women and 5 men during a 9 year period. Gastrointestinal bleeding due to ruptured esophageal varices revealed the liver disease in 11 cases. Hepatomegaly and splenomegaly were noted in 9 cases and ascites in 7. Anicteric cholestasis was demonstrated in 10 cases. Another disease, e. g. myelofibrosis and monoclonal gammapathy, was present in 11 patients. In 10 patients, portal diversion was performed; outcome being favorable with a follow-up of one to six years. The analysis of these cases and of the 113 previously published reports calls for the following comments: 1) In most cases, NRH is characterized by small-sized hepatocytic nodules scattered throughout the entire liver with no surrounding fibrosis; however this histological pattern may vary somewhat, with adjacent normal zones being found adjacent to typical cirrhotic fibrosis; although a precise morphometric study was not performed in our patients, obstruction of the tiny branches of intrahepatic portal veins was not observed. 2) Histological diagnosis of NRH is difficult and in most cases requires surgical biopsy specimens and specific coloration of the reticulin network. 3) NRH must be considered as a new cause of intrahepatic (sinusoidal or presinusoidal) portal hypertension and/or of chronic anicteric cholestasis. 4) A number of various conditions may be associated with NRH, the most frequent being Felty's syndrome and myeloproliferative disorders. 5) The pathogenesis of NRH remains unknown. 6) Portal diversion generally has a favorable outcome in this disease.     

Focal nodular hyperplasia of the liver. Five cases.

Focal nodular hyperplasia (FNH) of the liver is a benign tumour with a cirrhosis-like appearance. The tumour is rare, but it is a relevant differential diagnosis in young patients with a hepatic mass. Radiologically and grossly FNH cannot be distinguished from malignant tumours. Biopsy with frozen section usually provides the diagnosis. There is no evidence that FNH is premalignant. Only a few patients have symptoms requiring treatment (pain, loss of appetite, nausea, and vomiting) and complications (weight loss, portal compression) are extremely rare. The preferred treatment for symptomatic tumours is removal. In patients without symptoms FNH can be left untreated without any undue risk.     

Coexistence of nodular regenerative hyperplasia of the liver and pulmonary arterial hypertension in patients with connective tissue diseases: report of three cases and review of the literature

Abstract

Nodular regenerative hyperplasia of the liver (NRH) is known to be a rare condition in patients with connective tissue diseases (CTD). In this report, we document three patients with CTD who had both NRH and pulmonary hypertension (PH). All three patients developed PH during their course and thereafter developed NRH. The clinical course of these patients suggests that circulatory disturbance caused by PH might be a trigger for NRH.     

Familial occurrence of nodular regenerative hyperplasia of the liver: a report on three families

BACKGROUND/AIMS: Nodular regenerative hyperplasia of the liver is a histological lesion usually associated with systemic diseases, haematological malignancies, or drugs. Its prognosis depends on portal hypertension, which usually is well tolerated and requires medical management only. PATIENTS: Three unrelated families, in which two sibling adult male patients presented with nodular regenerative hyperplasia of the liver, were studied. METHODS: Complete clinical charts and liver biopsy specimens were available for all patients. In addition, explanted livers were available for examination for the two transplanted patients. RESULTS: There was no evidence of any of the various clinical situations known to be associated with nodular regenerative hyperplasia of the liver. Portal hypertension was severe, requiring surgical treatment in two cases. Renal lesions were present in three patients. In two patients, progressive evolution to liver atrophy and hepatic failure, associated with renal failure, led to combined liver and renal transplantation. CONCLUSIONS: This report describes the existence of familial cases of nodular regenerative hyperplasia of the liver, occurring without underlying or associated systemic disease, characterised by a poor clinical course and often associated with progressive renal failure.     

Coexistence of nodular regenerative hyperplasia of the liver and pulmonary arterial hypertension in patients with connective tissue diseases: report of three cases and review of the literature

Nodular regenerative hyperplasia of the liver (NRH) is known to be a rare condition in patients with connective tissue diseases (CTD). In this report, we document three patients with CTD who had both NRH and pulmonary hypertension (PH). All three patients developed PH during their course and thereafter developed NRH. The clinical course of these patients suggests that circulatory disturbance caused by PH might be a trigger for NRH.