Ischemic enteritis resulting from polycythemia vera.

Polycythemia vera is a chronic myeloproliferative disorder characterized by clonal proliferation of bone marrow progenitors. We report a case of ischemic enteritis and bowel obstruction resulting from polycythemia vera. A 76-year-old man was admitted to our hospital with abdominal distention. Contrast-enhanced computed tomography revealed portal vein thrombosis, superior mesenteric vein thrombosis, and dilated small intestinal loops with caliber changes at the end of the ileum. Laboratory data on admission revealed increased leukocyte, red blood cell, hemoglobin, and platelet levels. Polymerase chain reaction analysis for Janus kinase 2 V617F point mutation was positive. Intestinal obstruction due to either bowel adhesion or paralytic ileus secondary to the superior mesenteric artery and vein thrombosis caused by polycythemia vera was diagnosed. For decompression of the small intestinal obstruction, a transnasal ileus tube was placed. Despite conservative therapy, the small intestinal obstruction did not improve remarkably. Therefore, we decided to perform surgical treatment. Operative findings revealed extensive stricture in the ileum. Altogether, 30 cm of the ileum, including the known intestinal strictures, was resected. The pathological findings were consistent with ischemic enteritis. To the best of our knowledge, no case of ischemic enteritis caused by polycythemia vera has been previously reported in the literature.

The Administration of Tenofovir Disoproxil Fumarate for Pregnant Japanese Women with Chronic Hepatitis B.

The appropriate management of hepatitis B virus (HBV) infection during pregnancy has not been established in Japan. We herein report five HBV-infected pregnant Japanese women who received tenofovir disoproxil fumarate (TDF). Two of them had been born after the introduction of nationwide immunoprophylaxis and were vertically infected with HBV, highlighting the need to address mother-to-child transmission further. In both entecavir-experienced and nucleoside/nucleotide analog-naïve mothers, TDF suppressed HBV replication without serious adverse events. All five children were free from congenital disorders, growth impairment, and HBV infection. TDF showed safety and efficacy for pregnant woman with chronic hepatitis B and might have helped prevent mother-to-child transmission.

Effects on anemia of drug adjustment in patients with chronic hepatitis C during telaprevir-combined therapy.

AIM: Anemia is the most common adverse event in patients with chronic hepatitis C virus (HCV) treated with telaprevir (TVR) combined triple therapy. We examined the effects of drug dose adjustment on anemia and a sustained viral response (SVR) during combination therapy. MATERIAL AND METHODS: This study enrolled 62 patients treated with TVR (2,250 mg) for 12 weeks plus pegylated interferon-alpha-2b and ribavirin for 24 weeks. The patients were assigned randomly to the TVR-standard or -reduced groups before treatment. At the occurrence of anemia (hemoglobin < 12 g/dL), the TVR-reduced group received 1500 mg TVR plus the standard dose of ribavirin, whereas the TVR-standard group received the standard TVR dose (2,250 mg) and a reduced dose of ribavirin (200 mg lower than prescribed originally). The safety and SVR at 24 weeks were compared between the TVR-standard (n = 28) and TVR-reduced (n = 25) groups. RESULTS: No differences in the proportion of patients who became HCV RNA-negative were detected between the TVR-standard and -reduced groups (72 and 72% at week 4, 79 and 84% at the end of treatment, and 76 and 80% at SVR24, respectively). Two groups had comparable numbers of adverse events, which led to the discontinuation of TVR in 14 patients of TVR-standard group and in 14 of TVR-reduced group. A lower incidence of renal impairment was observed in the TVR-reduced group (6%) than the TVR-standard group (11%, not statistically significant). CONCLUSIONS: TVR dose adjustment could prevent anemia progression without weakening the anti-viral effect during triple therapy in HCV-patients.

[Combination Chemotherapy Using Sorafenib and Hepatic Arterial Infusion with a Fine-Powder Formulation of Cisplatin for Advanced Hepatocellular Carcinoma with Portal Vein Tumor Thrombosis--A Case Report].

Sorafenib has been a standard therapy for advanced hepatocellular carcinoma (HCC) with portal vein thrombosis. Hepatic arterial infusion chemotherapy (HAIC) is still preferably performed in Japan because of its relatively good tumor-shrinking effect. We report a case of advanced multiple HCC with portal thrombus that responded to combination chemotherapy with sorafenib and repeat hepatic arterial infusion with a fine-powder formulation of cisplatin (IA-call®). A 57-year-old man presented for the treatment of HCC with alcoholic cirrhosis. Multiple HCC were found to be rapidly progressing with portal thrombosis. HAIC with IA-call® was performed, but the tumors progressed. TAE was performed 3 times thereafter and the main tumor shrunk to some extent. A month after the last TAE, the HCC was found to progress again, and oral sorafenib was administered. A reservoir and catheter were placed and HAIC with low-dose 5-fluorouracil and cisplatin was performed for 3 cycles following 1 HAIC cycle with epirubicin and mitomycin C, which was not effective. For 10 months after initial therapy, HAIC using IA-call® has been performed once for 6 weeks. After performing HAIC with IA-call® 5 times, the serum levels of HCC tumor markers AFP and PIVKA-Ⅱdecreased, and the tumors continued to shrink and were not stained on enhanced CT scan. The patient has been alive for 23 months after the initial therapy and has maintained stable disease.

[A case of paralytic ileus associated with varicella zoster virus infection].

A 79-year-old woman with a history of pyothorax was admitted with a 4-day history of abdominal distension. Physical examination revealed marked abdominal distention, absent bowel sounds, and a vesicular rash over the left Th8-10 dermatome. Abdominal radiography showed gaseous distension of the colon and ileum. Colonoscopy excluded any obstructive process of the colon. Laboratory findings yielded positive results for serum IgM and IgG against the varicella zoster virus (VZV) . Paralytic ileus associated with the VZV was therefore diagnosed. The ileus improved after conservative treatment with intravenous acyclovir. Although shingles is frequently encountered, it is a rare cause of paralytic ileus. In the future, the VZV should be considered as one of the causes of paralytic ileus, and complete resolution can be achieved with conservative management.

[Three cases of recurrent hepatocellular carcinoma treated with laparoscopic hepatectomy after oral administration of sorafenib].

Case 1 involved a 63-year-old woman who had multiple hepatocellular carcinomas (HCC) associated with hepatitis C virus infection. She was treated with hepatic arterial infusion chemotherapy (HAIC) and transcatheter arterial chemoembolization (TACE), followed by oral administration of sorafenib (200 mg/day). However, a lesion on the left lateral segment of the liver enlarged rapidly. Laparoscopic left lateral sectionectomy was performed. Histopathological examination revealed combined hepatocellular and cholangiocellular carcinoma. Case 2 involved a 54-year-old man who had multiple recurrent HCCs associated with hepatitis C virus infection after undergoing hepatectomy twice. Oral administration of sorafenib (200 mg/day) was initiated. The intrahepatic lesions decreased in size, but metastasis in an enlarged lymph node behind the portal vein was suspected. Laparoscopic partial hepatectomy and lymph node dissection were performed. HCC metastasis in the lymph node and partial necrosis in the intrahepatic lesions were identified histopathologically. Case 3 involved a 56- year-old man who had multiple recurrent HCCs associated with hepatitis C virus infection. TACE was performed thrice, and oral administration of sorafenib( 400-600 mg/day) was initiated. The lesion in the right lateral segment of the liver decreased in size but lesions in the left lateral segment were enlarged. Therefore, laparoscopic left hemihepatectomy was performed.

[A case of primary biliary cirrhosis with systemic lymph node enlargement].

A 40's woman was hospitalized with cervical lymph node enlargement. Laboratory examinations showed elevated serum bile duct enzymes and the presence of anti-mitochondrial antibody. Abdominal ultrasonography and computed tomography showed enlargement of not only perihepatic lymph nodes, but also axillary and cervical lymph nodes. FDG-PET showed intense uptake concordant with these lymph nodes. We performed endoscopic ultrasonographic fine-needle aspiration biopsy of a perihepatic lymph node, but detected no malignant cells. We then performed liver biopsy, and obtained a histological diagnosed primary biliary cirrhosis. Systemic lymph nodes decreased together with serum bile duct enzyme levels during treatment with ursodeoxycholic acid.

Favorable factors for re-treatment with pegylated interferon α2a plus ribavirin in patients with high viral loads of genotype 1 hepatitis C virus.

AIM: Effect of re-treatment for pegylated interferon (PEG-IFN) plus ribavirin was not fully evaluated. We examined the effects of re-treatment with PEG-IFN plus ribavirin in patients with high viral loads of genotype 1 hepatitis C virus who failed to achieve a sustained virological response (SVR) with combination therapy. METHODS: We examined 38 patients who were re-treated with PEG-IFN α2a plus ribavirin for more than 60 weeks, among whom 14 were non-responders and 24 were relapsers after previous treatment with PEG-IFN α2b plus ribavirin. IL28B genotyping was done in 21 patients. RESULTS: The overall SVR rate was 34%. Analysis of baseline characteristics showed that the relapsers had a significantly higher SVR rate than the non-responders (50.0%, 12/24 vs. 7.1%, 1/14, respectively, P = 0.012) The SVR rates of re-treated patients who had turned hepatitis C virus (HCV) RNA-negative at weeks 8, 12, 24, and 48 of the previous therapy were 67% (4/6), 67% (4/6), 29% (2/7), and 25% (1/4), respectively. Re-treatment achieved an SVR in five of 12 patients with IL28B major alleles and three of nine patients with IL28B minor alleles. During the re-treatment, patients with complete viral suppression at week-12 achieved a significantly higher SVR rate (P = 0.001). CONCLUSIONS: Re-treatment with PEG-IFN α2a plus ribavirin therapy is effective in patients who relapse after a course of PEG-IFN α2b plus ribavirin therapy. Re-treatment is a particularly useful option for patients who achieve early viral clearance during previous therapy.

A case of Wegener's granulomatosis associated with progressive dysphagia owing to esophageal involvement.

A 52-year-old woman was admitted to our hospital with progressive dysphagia. Upper gastrointestinal endoscopy revealed esophageal stenosis and computed tomographic scan revealed symmetrical wall thickness of the thoracic esophagus. Biopsies findings from a lesion were unremarkable. However, a definitive diagnosis of Wegener's granulomatosis was based on positive anti-neutrophil cytoplasmic antibodies directed against proteinase 3 and otorhinolaryngological manifestations. Esophageal complications are rarely reported in Wegener's granulomatosis; however, clinicians should be aware of the possibility of esophageal involvement.

A case of mesenteric lymphadenitis with long-acting symptom, showing marked response to corticosteroid.

A 17-year-old girl who had right lower abdominal pain with multiple swelling of lymph nodes in the ileocecal region and she was admitted to a neighboring hospital. Since there was no improvement of condition with antibiotic resistance, we were consulted. Lymph node biopsy under laparoscopy demonstrated nonspecific findings. She then developed erythema nodosum, suggesting the presence of autoimmune etiology. Treatment with systemic corticosteroid resulted in symptomatic improvement. Mesenteric lymphadenitis like this case is rare.

Spontaneous regression of hepatocellular carcinoma: report of a case.

A spontaneous regression of hepatocellular carcinoma is an extremely rare phenomenon. A 69-year-old Japanese man with hepatitis C virus-related chronic hepatitis presented with a liver tumor. We diagnosed the tumor to be hepatocellular carcinoma in the course of spontaneous regression, by imaging studies and changes in the tumor markers. Because the possible presence of viable cancer cells could not be ruled out, we recommended surgery. He refused all treatments at first, but finally agreed to undergo surgery about 10 months after presentation. A hepatectomy was performed. Histologically, no viable tumor cells were found. In our case, the vascularity of the tumor according to the imaging findings was followed up during the clinical course. The patient is now doing well and without any evidence of recurrence at 37 months after surgery.