Case of autoimmune hepatitis with markedly enlarged hepatoduodenal ligament lymph nodes.

Autoimmune hepatitis (AIH) is a necroinflammatory liver disease of unknown etiology. The disease is characterized histologically by interface hepatitis, biochemically by increased aspartate aminotransferase and alanine aminotransferase levels, and serologically by increased autoantibodies and immunoglobulin G levels. Here we discuss AIH in a previously healthy 37-year-old male with highly elevated serum levels of soluble interleukin-2 receptor and markedly enlarged hepatoduodenal ligament lymph nodes (HLLNs, diameter, 50 mm). Based on these observations, the differential diagnoses were AIH, lymphoma, or Castleman's disease. Liver biopsy revealed the features of interface hepatitis without bridging fibrosis along with plasma cell infiltration which is the typical characteristics of acute AIH. Lymph node biopsy revealed lymphoid follicles with inflammatory lymphocytic infiltration; immunohistochemical examination excluded the presence of lymphoma cells. Thereafter, he was administered corticosteroid therapy: after 2 mo, the enlarged liver reached an almost normal size and the enlarged HLLNs reduced in size. We could not find AIH cases with such enlarged lymph nodes (diameter, 50 mm) in our literature review. Hence, we speculate that markedly enlarged lymph nodes observed in our patient may be caused by a highly activated, humoral immune response in AIH.

Factors associated with the overall survival of elderly patients with hepatocellular carcinoma.

AIM: To identify the factors associated with overall survival of elderly patients with hepatocellular carcinoma (HCC). METHODS: A total of 286 patients with HCC (male/female: 178/108, age: 46-100 years), who were diagnosed and treated by appropriate therapeutic procedures between January 2000 and December 2010, were enrolled in this study. Patients were stratified into two groups on the basis of age: Elderly (≥ 75 years old) and non-elderly (< 75 years old). Baseline clinical characteristics as well as cumulative survival rates were then compared between the two groups. Univariate and multivariate analyses were used to identify the factors associated with prolonged overall survival of patients in each group. Cumulative survival rates in the two groups were calculated separately for each modified Japan Integrated Stage score (mJIS score) category by the Kaplan-Meier method. In addition, we compared the cumulative survival rates of elderly and non-elderly patients with good hepatic reserve capacity (≤ 2 points as per mJIS). RESULTS: In the elderly group, the proportion of female patients, patients with absence of hepatitis B or hepatitis C viral infection, and patients with coexisting extrahepatic comorbid illness was higher (56.8% vs 31.1%, P < 0.001; 27.0% vs 16.0%, P = 0.038; 33.8% vs 22.2%, P = 0.047; respectively) than that in the non-elderly group. In the non-elderly group, the proportion of hepatitis B virus (HBV)-infected patients was higher than that in the elderly group (9.4% vs 0%, P = 0.006). The cumulative survival rates in the elderly group were 53.7% at 3 years and 32.9% at 5 years, which were equivalent to those in the non-elderly group (55.9% and 39.4%, respectively), as shown by a log-rank test (P = 0.601). In multivariate analysis, prolonged survival was significantly associated with the extent of liver damage and stage (P < 0.001 and P < 0.001, respectively), but was not associated with patient age. However, on individual evaluation of factors in both groups, stage was significantly (P < 0.001) associated with prolonged survival. Regarding mJIS scores of ≤ 2, the rate of female patients with this score was higher in the elderly group when compared to that in the non-elderly group (P = 0.012) and patients ≥ 80 years of age tended to demonstrate shortened survival. CONCLUSION: Survival of elderly HCC patients was associated with liver damage and stage, but not age, except for patients ≥ 80 years with mJIS score ≤ 2.

Adenoendocrine cell carcinoma of the gallbladder producing a high level of alpha-fetoprotein.

We present the case of a 78-year-old Japanese woman with adenoendocrine cell carcinoma of the gallbladder accompanied by a high serum alpha-fetoprotein level. The patient visited our hospital with a complaint of a large mass in the right hypochondrium. Ultrasonography and computed tomography revealed multiple large hepatic tumors, swelling of lymph nodes in the hepatic hilum and para-aortic regions, and a slightly irregular gallbladder wall. The serum alpha-fetoprotein level was 157,428 ng/mL. We initially suspected scirrhous hepatocellular carcinoma, sarcomatous hepatocellular carcinoma, biliary tract cancer, or pancreatic cancer. However, the hepatic tumor biopsy was histologically diagnosed as undifferentiated adenocarcinoma. Immunohistochemical analysis demonstrated that the tumor was positive for cytokeratin 19, focally positive for cytokeratin 7, but negative for hepatocyte paraffin 1 and cytokeratin 20, suggestive of biliary tract carcinoma. Although the patient received a course of hepatic arterial infusion chemotherapy with cisplatin, she died 2 months after admission. Histopathological examination at autopsy revealed that the hepatic tumor was adenoendocrine cell carcinoma of the gallbladder, which was positive for cytokeratin 19, focally positive for cytokeratin 7, chromogranin A, synaptophysin, and weakly positive for alpha-fetoprotein. Labeling index of Ki-67 was 28 %. Interestingly, this was the first case report of adenoendocrine cell carcinoma of the gallbladder that produced a high level of alpha-fetoprotein, which hampered correct diagnosis before autopsy.

Relapse of hepatitis C in a pegylated-interferon-alpha-2b plus ribavirin-treated sustained virological responder.

A 41-year-old woman with chronic hepatitis C was treated with pegylated-interferon (PEG-IFN)-alpha-2b plus ribavirin for 24 weeks. She had hepatitis C virus (HCV) genotype 2a (1600 KIU/mL), and her liver histology showed mild inflammation and fibrosis. Four weeks after the start of the therapy, she achieved a rapid virological response (RVR) and then a sustained virological response (SVR). Serum alanine aminotransferase (ALT) levels remained within normal ranges and HCV RNA continued to be negative. However, ALT levels flared with the re-emergence of HCV RNA in the serum 1.5 years after discontinuation of therapy. HCV RNA obtained from sera before therapy and after relapse shared a 98.6% homology with the E2 region, and phylogenetic analyses indicated that they were the same HCV strain. These results eliminated the possibility of a re-infection and strongly indicated a late relapse of the disease. Therefore, follow-up is necessary for chronic hepatitis C patients after SVR, even if they respond well to therapy, including RVR.

Non-prescription supplement-induced hepatitis with hyperferritinemia and mutation (H63D) in the HFE gene.

A 55-year-old Japanese woman was hospitalized because liver function tests showed an abnormality. Transaminases and biliary enzymes were markedly elevated with hyperferritinemia. Her imaging tests revealed no significant abnormality. She had been taking various non-prescription supplements for over approximately 6 months. After the supplements were discontinued her liver function gradually improved. This clinical course was suggestive of supplement-induced hepatitis. She had no history of taking supplements containing iron, so it was interesting that she had hyperferritinemia. We examined C282Y and H63D, which are important mutations in theiron-metabolizing gene, HFE. She was found to be heterozygous for the H63D mutation. The interaction between hyperferritinemia and supplements is unknown, but it can be speculated that some interaction between iron overload and supplements may have underlain the pathogenesis of her liver injury.

Clinicopathological analysis of a case with mesenteric solitary Castleman's disease: diagnostic value of radiological findings.

We report a case with rare solitary mesenteric Castleman's disease (CD). A 45-year-old woman complaining of nausea was presented. A round-shaped, smooth margin, and hypoechoic mass was seen on screening abdominal ultrasonography. Computed tomography showed a markedly enhanced tumor anterior to the left iliopsoas muscle. Selective jejunal arteriography revealed an extreme hypervascularity without vascular invasion. These results retrospectively seem to differ from those of malignant lymphoma or sarcoma. The tumor was surgically resected, and hyaline-vascular type CD was pathologically diagnosed. We postulate that these radiological findings might suggest hyaline-vascular type CD as one of the differential diagnoses in similar cases, although more clinical data should be evaluated.

[A case of recrudescent groove pancreatitis, which was in remission by proximal gastrectomy with vagotomy for gastric cancer].

This report describes our experience with a 60 year old male who suffered from a recrudescence of groove pancreatitis. He had been treated by conservative medication therapy by proton pump inhibitor used for therapy of duodenal ulcer, and was in remission. During a follow-up one year later, endoscopy revealed gastric cancer, for which a proximal gastrectomy and vagotomy were performed. The patient continues to remain in remission for the groove pancreatitis. Our experience with the clinical course of this disease, in which treatment for duodenal ulcer was used effectively, offers new insights into the progression and therapy of groove pancreatitis.