OVERVIEW
Nodular regenerative hyperplasia of the liver (NRH) is a chronic non-sclerotic liver disease characterized by diffuse regenerative nodules less than 3 cm in diameter in the hepatic parenchyma, with no or only mild liver fibrosis. It can occur in patients of any age, often presents clinically with portal hypertension, is often associated with many extrahepatic chronic diseases, is often combined with immune or hematologic disorders, and is histopathologically characterized by hepatocellular nodule formation in the liver parenchyma with mild fibrosis.
Etiology
The cause of the disease is unknown, and it is associated with systemic diseases, such as systemic lupus erythematosus, rheumatoid arthritis, desiccation syndrome, Felty syndrome, polyarteritis nodosa, progressive systemic sclerosis, antiphospholipid antibody syndrome and other autoimmune disorders, as well as idiopathic thrombocytopenic purpura, true erythrocytosis, macroglobulinemia, myelofibrosis, plasma cell disorders, leukemia, lymphoma, and other diseases. disease, leukemia, lymphoma, and other hematologic disorders. It is also seen in people with a history of medication such as glucocorticoids, immunosuppressants, and cytotoxic drugs, or in people with genetic abnormalities such as portal hypoplasia or congenital heart disease.
Symptoms
Some patients present with abdominal distension and abdominal pain; symptoms associated with portal hypertension, such as esophageal varices, ascites, and gastrointestinal bleeding; and hepatic single or multiple occupations and concurrent immune and hematologic manifestations, such as fever, rash, arthritis, Raynaud’s phenomenon, proteinuria, hepatosplenomegaly, and lymph node enlargement. In a few patients, nodule rupture may occur due to the obvious enlargement of nodules in the liver, thus manifesting as acute abdominal pain, hemoperitoneum, hemorrhagic shock, or even death.
Examination
Liver puncture biopsy and surgical histopathologic biopsy are mainly performed. The histopathologic feature of this disease is diffuse nodular changes in the liver.
Diagnosis
Liver puncture biopsy is difficult to confirm the diagnosis of this disease, and mainly relies on surgical histopathologic biopsy. Some patients do not have clinical symptoms or signs of liver disease, but are only found during physical examination or related tests for other diseases, and some are diagnosed by biopsy during surgery when abnormal changes are found in the liver.
Treatment
Systemic diseases need to be treated if hepatic nodular aplasia is combined with systemic diseases. Surgery is required for portal hypertension and space-occupying lesions of the liver. If nodular aplasia progresses to end-stage liver disease and liver failure occurs, liver transplantation is required.
Prognosis
Depending on the severity of portal hypertension, the efficacy of diagnosis and treatment, and the severity of combined systemic diseases, the prognosis of the disease is better than that of cirrhosis if the systemic diseases are well controlled. The prognosis of patients with hepatic space without portal hypertension is better.