A Case of Sclerosing Angiomatoid Nodular Transformation of the Spleen: Spoke Wheel Pattern on Computed Tomography / 대한내과학회지

Sclerosing angiomatoid nodular transformation (SANT) is a rare, benign vascular neoplasm. Most patients have no clinical symptoms, and the tumors are usually discovered incidentally on abdominal computed tomography or ultrasonography. Some studies have reported the clinical features and imaging findings of SANT, but the diagnosis is based on histopathologic examination of a tissue specimen obtained at splenectomy. We report herein an incidentally discovered case of SANT and review the related literature.

Serum transferrin as a liver fibrosis biomarker in patients with chronic hepatitis B

BACKGROUND/AIMS: Transferrin and alpha-1 antitrypsin are reportedly associated with liver fibrosis. We evaluated the usefulness of serum transferrin and alpha-1 antitrypsin as new liver fibrosis markers in patients with chronic hepatitis B. METHODS: The study included 293 patients with chronic hepatitis B who underwent a liver biopsy between October 2005 and June 2009, and who had no history of hepatocellular carcinoma. Serum markers and liver fibrosis stages were compared. RESULTS: Univariate analysis revealed that age (P<0.001), serum platelet count (P<0.001), and serum alkaline phosphatase level (P=0.003) differed significantly between the patients with and without liver cirrhosis. Serum transferrin levels were significantly lower in advanced fibrosis than in mild fibrosis in both univariate analysis (P=0.002) and multivariate analysis (P=0.009). In addition, the serum transferrin level was significantly lower in cirrhotic patients than in noncirrhotic patients (P=0.020). However, the serum level of alpha-1 antitrypsin was not significantly associated with liver cirrhosis in patients with chronic hepatitis B. CONCLUSIONS: Serum transferrin could be promising serum marker for predicting advanced liver fibrosis in patients with chronic hepatitis B.

Etanercept for steroid-refractory acute graft versus host disease following allogeneic hematopoietic stem cell transplantation

BACKGROUND/AIMS: The treatment for steroid-refractory acute graft versus host disease (GVHD) after allogeneic stem cell transplantation (allo-SCT) needs to be standardized. We report our clinical experience with etanercept for steroid-refractory acute GVHD. METHODS: Eighteen patients who underwent allo-SCT and presented with steroid-refractory acute GVHD at Ajou University Hospital were studied retrospectively. They were given 25 mg of etanercept subcutaneously twice weekly for 4 weeks. The clinical responses were evaluated with regard to the severity of acute GVHD. RESULTS: The median patient age was 43.5 years. Using nonparametric tests, etanercept had a down-grading effect on acute GVHD (p = 0.005), although no patient experienced complete remission. Partial responses were seen in 80%, 17%, and 57% of grade II to IV patients, respectively. Skin and gut GVHD were well controlled with etanercept, whereas hepatic GVHD was not. Four patients died of fatal infections. No factors affecting the clinical outcome of etanercept were identified. CONCLUSIONS: Etanercept has a modest effect on steroid-refractory acute GVHD after allo-SCT, with tolerable side effects.

A Case of Multiple Myeloma Presenting Acute Renal Failure in a Patient with Rheumatoid Arthritis

It is known that rheumatoid arthritis (RA) patients show increased incidence of multiple myeloma (MM), despite its rarity. Only one case of MM with seronegative RA was reported in Korea, thus far. We report a case of MM with seropositive RA. The patient was a 66 year old female who had been diagnosed with seropositive RA 4 years ago. Over the last 1 month, the patient experienced general weakness and weight loss of 10 kg. It was found that her serum creatinine had increased and her urine analysis showed proteinuria. To evaluate renal failure and proteinuria, renal biopsy, bone marrow biopsy and electrophoresis were carried out. A diagnosis of myeloma cast nephropathy was made. We report this rare case of MM represented as acute renal failure during the treatment for RA, and include a review of the literature.

Kartagener's syndrome with immunoglobulin G subclass deficiency

Kartagener syndrome is characterized by the triad of situs inversus, bronchiectasis, and chronic paranasal sinusitis. Recurrent sinopulmonary infection, the major determinant for diagnosing immunodeficiency, is the most common clinical manifestation of the disease. A 17-year-old female patient presented with dyspnea, cough, sputum, nasal congestion, and rhinorrhea for more than 5 years. Nasal symptoms and dyspnea had not been controlled by intermittent treatment with mucolytics and antibiotics from primary clinics since 3 months before visiting our clinic. Chest X-ray and computed tomography showed situs inversus, dextrocardia and bronchiectasis. Paranasal sinus series revealed mucosal thickening and haziness on both maxillary sinus. Serum immunoglobulin (Ig) G4 was decreased, but total IgG was within normal range. Under the diagnosis of Kartagener syndrome with IgG4 deficiency, monthly intravenous IgG (IVIG) treatment was performed for 6 months. Her symptoms were well controlled and the frequency of antibiotics use was markedly decreased. We report a patient having the Kartagener syndrome with IgG4 deficiency that was successfully controlled with a 6-month-treatment of IVIG.