Autoimmune hepatitis complicated by adult-onset Still's disease during treatment with tocilizumab: A case report from acute onset to recurrence.

The characteristics of liver dysfunction due to adult-onset Still's disease are not specific. Differentiating from autoimmune hepatitis is important in deciding whether to continue corticosteroid therapy, and also in terms of management of cirrhosis and surveillance of hepatocellular carcinoma. Liver biopsy is thought to be the most important determinant for differential diagnosis.

Intestinal Behçet's disease complicated by myelodysplastic syndrome and secondary pulmonary alveolar proteinosis: a case report.

BACKGROUND: Gastrointestinal lesions, which sometimes develop in Behçet's disease (BD), are referred to as intestinal BD. Although rare, intestinal BD can be accompanied by myelodysplastic syndrome (MDS) with abnormal karyotype trisomy 8, which is refractory to immunosuppressive therapy. Pulmonary alveolar proteinosis is a rare lung complication of BD and MDS. Herein, we present an extremely rare case of intestinal BD presenting with MDS and several chromosomal abnormalities, followed by secondary pulmonary proteinosis. CASE PRESENTATION: A 58-year-old Japanese woman with a 3-year history of genital ulcers and oral aphthae was admitted to our hospital. The patient developed abdominal pain and persistent diarrhea. Colonoscopy revealed multiple, round, punched-out ulcers from the terminal ileum to the descending colon. Intestinal BD was diagnosed and the patient was treated with colchicine, prednisolone, and adalimumab. However, her symptoms were unstable. Bone marrow examination to investigate the persistent macrocytic anemia revealed the presence of trisomy 8, trisomy 9, and X chromosome abnormalities (48, + 8, + 9, X, i(X) (q10) in 12 out of the examined 20 cells). Based on her hypoplastic bone marrow, the patient was diagnosed with low-risk MDS (refractory anemia). At the age of 61, the patient developed pneumonia with fever and diffuse ground-glass opacities on the lung computed tomography (CT). Chest high-resolution CT and histopathology via transbronchial lung biopsy revealed the presence of pulmonary alveolar proteinosis (PAP). These findings combined with the underlying disease led to the diagnosis of secondary PAP. CONCLUSIONS: Secondary pulmonary proteinosis may accompany intestinal BD with MDS and several chromosomal abnormalities. Physicians should pay attention to lung complications, such as PAP, in patients with intestinal BD complicated by MDS. Genetic abnormalities may be associated with the development of such diseases.

[Adult case of hypoganglionosis of the colon with a skip lesion: a case report].

A 77-year-old woman was referred for severe constipation and abdominal distension which had lasted for 1 month. A computed tomography (CT) scan and a colonoscopy revealed segmental stricture in the transverse and descending colons. After no improvement in her symptoms was observed with conservative therapy, we performed a left hemicolectomy. Histopathological examination revealed a reduction in ganglion cells in the Meissner's and Auerbach's plexuses and hypoganglionosis was diagnosed. In addition, a diagnosis of acquired hypoganglionosis was made because this case was adult onset, and there has been no recurrence.

Inflammatory Hepatocellular Adenoma with Elevated Serum Protein Induced by Vitamin K Absence/Antagonist-II in Adult Males.

Two men (24 and 34 years of age) with a single hypervascular liver tumor were admitted to our hospital. The tumors were diagnosed as hepatocellular adenoma (HCA) by an ultrasound-guided biopsy and classified as inflammatory type by immunohistochemical staining. Considering the risk of malignant transformation, they underwent surgical resection. Although the serum levels of protein induced by vitamin K absence/antagonist-II (PIVKA-II) were slightly elevated, they normalized after the resection. The diagnosis of HCA including malignant transformation is often difficult by image findings alone. Careful immunohistochemical examinations are very useful for the diagnosis and classification of subgroups, including malignant transformation. In addition, we proved that HCA without malignant transformation expresses PIVKA-II.

[Hemobilia caused by hepatic artery pseudoaneurysm after endoscopic biliary stenting for malignant hilar biliary strictures:a case report].

A 78-year-old female presented at our hospital with hilar biliary strictures caused by gallbladder cancer. Metal stents with a dilated diameter of 8mm were placed in a side-by-side fashion in the left and right hepatic ducts. However, 3 months after stenting, the patient experienced a sudden onset of hematemesis. Emergent enhanced abdominal angiography revealed a right hepatic arterial pseudoaneurysm that had likely ruptured, thus causing the hemobilia. Probable association of biliary stents with pseudoaneurysm was also demonstrated. Selective angiography revealed bleeding from the pseudoaneurysm into the biliary stents, which was controlled by coil embolization. The patient was subsequently discharged on hospital day 15.

Spontaneous Regression of Hepatocellular Carcinoma with Portal Vein Tumor Thrombus.

An 83-year-old man underwent transcatheter arterial chemoembolization (TACE) for a 20-mm hepatocellular carcinoma (HCC) in Couinaud's segment 4. Computed tomography (CT) 4 months after TACE showed tumor thrombus in the portal vein in addition to diffuse metastases and arterioportal shunts in the left lobe. Although we performed the best supportive care, the tumor thrombus in the portal vein and tumors in the left lobe had completely disappeared on CT 16 months after the TACE. Rapidly grown portal vein tumor thrombus and arterioportal shunt might be the causes of spontaneous regression of HCC, probably associated with tumor hypoxia.

Successful chemotherapy with modified FOLFIRINOX for pancreatic acinar cell carcinoma.

Abdominal ultrasonography revealed a pancreatic mass in a 67-year-old man with diabetes mellitus. Endoscopic ultrasound-guided fine needle aspiration led to the histological diagnosis of acinar cell carcinoma. The clinical stage was determined to be IVb based on findings of multiple metastatic lesions in the liver and lymph nodes, as well as splenic vein infiltration. Because the patient was not a surgical candidate, he underwent chemotherapy with modified FOLFIRINOX. In the absence of any severe adverse events, 12 courses of chemotherapy were delivered, resulting in marked shrinkage of both the primary and metastatic lesions. The outcome was judged to be a partial response, which was maintained even 9 months from the introduction of the chemotherapy. The results of this case suggest that modified FOLFIRINOX is safe and effective in the treatment of pancreatic acinar cell carcinoma.

[Nonalcoholic steatohepatitis after pancreatoduodenectomy with rapid progression of hepatic fibrosis: a case report].

A 64-year-old woman who had undergone pancreatoduodenectomy for intraductal papillary mucinous carcinoma 10 months previously was referred to our department complaining of ascites and general malaise. Abdominal computed tomography (CT) showed a markedly decreased hepatic CT value. Liver biopsy revealed nonalcoholic steatohepatitis. Treatment with nutritional control and pancreatic enzyme supplements improved liver function. Exocrine pancreatic enzyme insufficiency from chronic pancreatitis is considered to be a cause of rapid progression of hepatic steatosis.

[A case of liver metastasis occurring 20 years after the resection of an ileal neuroendocrine tumor].

A 70-year-old man with multiple liver tumors was referred to our hospital in 2011. He was histologically diagnosed with a neuroendocrine G1 tumor (World Health Organization classification) following biopsy. He had a history of surgery for an ileal neuroendocrine tumor in 1991. Therefore, the liver tumors were diagnosed as metastases from the ileal neuroendocrine tumor. The patient was successfully treated with hepatic artery embolization, radiofrequency ablation, and octreotide. This report suggests that long-term follow-up with diagnostic imaging may be required for patients with ileal neuroendocrine tumors, even 20 years after the primary surgery.

Case of presymptomatic aceruloplasminemia treated with deferasirox.

Aceruloplasminemia is an autosomal recessive disease characterized by an abnormal iron metabolism. The absence of ferroxidase activity caused by mutation of ceruloplasmin leads to iron overload in the brain, liver and other organs. We report a 35-year-old man who was diagnosed with aceruloplasminemia without neurological manifestation despite the accumulation of iron in the brain and liver. To prevent the development of neurodegenerative disorder related to iron toxicity, iron depletion therapy was performed. Iron chelator deferasirox was effective in reducing serum ferritin level and to prevent the progression of the disease.