Monosomal and complex karyotypes as prognostic parameters in patients with International Prognostic Scoring System higher risk myelodysplastic syndrome treated with azacitidine

BACKGROUND: Azacitidine (AZA) is standard care for patients with myelodysplastic syndrome (MDS) who have not had allogeneic stem cell transplantation. Chromosomal abnormalities (CA) including complex karyotype (CK) or monosomal karyotype (MK) are associated with clinical outcome in patients with MDS. METHODS: We investigated which prognostic factors including CAs would predict clinical outcomes in patients with International Prognostic Scoring System (IPSS) higher risk MDS treated with AZA, retrospectively. CK was defined as the presence of three or more numerical or structural CAs. MK was defined as the presence of two or more distinct autosomal monosomies or single autosomal monosomy with at least one additional structural CA. RESULTS: A total of 243 patients who treated with AZA, were enrolled. CK was present in 124 patients and MK was present in 90 patients. Bone marrow blasts > or =15% and CK were associated with poorer response (P=0.038, P=0.007) and overall survival (OS) (P3 CAs was associated with poorer OS (group including 3 CAs vs. only three CAs, P=0.001). CONCLUSION: CK was an important prognostic parameter associated with worse outcome. MK may predict poor survival in only non-CK status. The higher number of CAs was associated with poorer survival.

A Common Bile Duct Web in Association with Bile Duct Stone Treated with Balloon Dilatation / 대한췌담도학회지

Bile duct web is very rare disease and it's etiology is controversial. Some webs are occurred in the presence of chronic inflammation, frequently associated with bile duct stone, but others are thought to be congenital. Many patients with bile duct web are asymptomatic, but they sometimes present symptom of biliary obstruction and cholangitis. It can be diagnosed by endoscopic retrograde cholangiopancreatogram, typically appearing as thin and shelf like radiolucent ring. We report a case of the common bile duct web with bile duct stones diagnosed by Endoscopic retrograde cholangiopancreatography (ERCP) in a 65-year-old man. The patient was treated by balloon dilatation successfully.

A Case of Intrahepatic Bile Duct Sarcoma; Misdiagnosed with Liver Abscess Combined with Intrahepatic Ductal Stone / 대한췌담도학회지

Sarcoma is rare malignant tumor originated from mesenchymal stem cells that can differentiate to soft tissue and bone. Therefore sarcoma can be arised from any regions in human body. However, the incidence of bile duct sarcoma is extremely rare in adults. Obstructive jaundice is the most common presentation in the patients, but there're no specific symptoms or signs. Also it can be misdiagnosed with other tumors or benign lesions in computed tomography or ultrasonography. For these reasons, it is hard to diagnose and manage. We present a case of intrahepatic bile duct sarcoma misdiagnosed with liver abscess in 70 year-old female with literature review.

Pancreatoblastoma in an Adult / 대한췌담도학회지

A blastoma is a type of cancer, which is common in children; it is caused by malignancies derived from in the precursor cells, often called blasts. Examples are nephroblastomas, retinoblastomas, pleuropulmonary blastomas, and pancreatoblastomas. Pancreatoblastomas are extremely rarely in adults. It is difficult preoperatively to distinguish this tumor from other pancreatic tumors including solid and papillary epithelial neoplasm of the pancreas (SPEN), acinar cell carcinoma, islet cell tumor, and ductal adenocarcinoma with cystic degeneration. To our knowledge, this case may be the second report of a pancreatoblastoma occurring in an adult in Korea. We report a case of a pancreatoblastoma that was confirmed by pathology, despite the radiologic finding that assumed it was a SPEN.

Association between Vitamin D Deficiency and Carotid Intima-media Thickness in Patients with Rheumatoid Arthritis

OBJECTIVE: The present study determined if vitamin D deficiency is a potential risk factor for increased carotid intima-media thickness (CIMT) in patients with rheumatoid arthritis (RA). METHODS: This cross-sectional study analyzed 50 consecutive female RA patients without cardiovascular disease history at the Pusan National University Hospital between September and December of 2013. CIMT was measured using a high-resolution ultrasonography. Serum 25-hydroxy vitamin D (25-OHD) levels were assessed by radioimmunoassay, and vitamin D deficiency was defined as serum 25-OHD levels <20 ng/mL. Stepwise multivariable linear regression analyses were performed to evaluate the association between vitamin D deficiency and increased CIMT. RESULTS: The median 25-OHD level (inter-quartile range) was 14.0 (11.0~20.7) ng/mL, and 74% of patients had vitamin D deficiency. The mean+/-standard deviation of CIMT was 0.58+/-0.08 mm. RA patients with vitamin D deficiency had significantly higher CIMT than those without this feature (0.59+/-0.07 vs 0.54+/-0.05, p=0.028). In univariable linear regression models, vitamin D deficiency (beta(SE)=0.047 (0.021), p=0.028), older age (beta(SE)=0.003 (7.2(-4)), p<0.001) and higher disease activity score 28-erythrocyte sedimentation rate (beta(SE)=0.021 (0.010), p=0.034) and Korean version of health assessment questionnaire score (beta(SE)=0.051 (0.015), p=0.002) were significantly associated with increased CIMT. Vitamin D deficiency remained statistically significant in multivariable regression models after adjusting for confounders. CONCLUSION: Vitamin D deficiency was associated with increased CIMT in female RA patients. Our finding suggests that hypovitaminosis D can be a risk factor for atherosclerosis in RA patients.