Allogeneic hematopoietic cell transplantation in adult patients with myelodysplastic/myeloproliferative neoplasms

BACKGROUND: In adults, the 2 main types of myelodysplastic/myeloproliferative neoplasms (MDS/MPN) are chronic myelomonocytic leukemia (CMML) and atypical chronic myeloid leukemia (aCML). Both are associated with a poor prognosis. Allogeneic hematopoietic cell transplantation (HCT) is the only known curative treatment modality for these diseases, but data on outcomes following such treatment are limited. We analyzed the outcomes of patients with MDS/MPN after allogeneic HCT. METHODS: This retrospective study included 10 patients with MDS/MPN who received allogeneic HCT at Asan Medical Center from 2002 to 2010. Of these 10 patients, 7 had CMML, 2 had aCML, and 1 had unclassifiable MDS/MPN. Five patients received a myeloablative conditioning (MAC) regimen (busulfan-cyclophosphamide), and 5 received reduced-intensity conditioning (RIC) regimen. RESULTS: Neutrophil engraftment was achieved in all patients. After a median follow-up of 47.5 months among surviving patients, 4 had relapsed and 5 had died. There was only 1 treatment-related death. The 5-year rates of overall, relapse-free, and event-free survival were 42.2%, 51.9%, and 46.7%, respectively. Relapse was the leading cause of treatment failure, and all relapses were observed in patients who had received RIC and who did not develop chronic graft-versus-host disease. CONCLUSION: Allogeneic HCT can induce durable remission in patients with MDS/MPN, but RIC cannot replace MAC in patients eligible for myeloablative treatments.

A Case of Hemophagocytic Syndrome as a Complication of Acute Hepatitis A / 대한내과학회지

Virus-associated hemophagocytic syndrome (VAHS) is a severe hematological disease characterized by persistent fever, peripheral blood cytopenia, hyperferritinemia, hypertriglyceridemia, and hemophagocytosis in the bone marrow, spleen, or lymph nodes. Despite the high incidence of viral infections in the general population, VAHS complicated by hepatitis A infection is rare worldwide, and there is no standard treatment for VAHS. Treatments include correcting the peripheral blood cytopenia and controlling the infectious causes and immunosuppression, such as with cyclosporine A and corticosteroids. We report on a patient who was diagnosed with hepatitis A virus-associated VAHS, and has been free of signs and symptoms of VAHS for 2 years with a combination of corticosteroid and cyclosporin A.

The Expression of TGF-beta Isoform mRNA in the Cataract Lens Epithelial Cell

PURPOSE: Transforming growth factor (TGF)-beta is recognized as being associated with cataractogenesis. We quantitated the mRNA expression of TGF-beta isoforms in cataractous crystalline lens to determine the effect of the isoforms on cataractogenesis. METHODS: With lens epithelial cells from thirty eyes in thirty patients, the mRNA expressions of TGF-beta1, beta2 and beta3 were quantitated by real-time SYBR Green polymerase chain reaction and the results were compared according to cataract type and presence of diabetes mellitus. RESULTS: Each isoform mRNA of TGF-beta was expressed: TGF-beta3 in all 30 eyes, TGF-beta1 in 29 eyes (96.7%), with the exception being one diabetic senile cataract, and TGF-beta2 in 9 eyes. The amount of TGF-beta1 mRNA expression was significantly lower in the diabetic cataracts than in the non-diabetic cataracts (P=0.056). CONCLUSIONS: TGF-beta was associated with cataractogenesis. It is significant that the expression of TGF-beta2 mRNA was decreased in all cataracts. The decrease of TGF-beta1 mRNA expression was more meaningful in the diabetic cataracts than in the non-diabetic cataracts.

28 cases of primary sclerosing cholangitis / 대한내과학회지

Backgroud : Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic hepatobiliary disease of unknown etiology, a characterized by inflammation, scarring and obliteration of bile ducts, eventually leading to biliary cirrhosis and liver failure. In Korea, reports on PSC have only been done anecdotally and there are hardly any large scale studies. METHODS: We retrospectively analyzed the clinical, laboratory, radiologic and histologic feature of 28 patients, who were diagnosed in Asan Medical Center during the last decade. A diagnosis of PSC was made by typical direct cholangiographic findings. RESULTS: The median age of the enrolled patients at diagnosis was 39.3 years, and there was a preponderance of men (M:F = 2.1:1). The major clinical features were abdominal pain and abnormal liver function tests of cholestatic pattern. The rate of positivity for autoantibody was 30.8% (8/26). On cholangiogram, 82.1% had evidence of both extra- and intrahepatic bile duct involvement, 10.7% of intrahepatic involvement only, and 7.1% of hilar involvement only. The pancreatic duct involvement was seen in 10.5% (2/19). The incidence of associated inflammatory bowel disease was 50% (6/12), UC in 5 cases and Crohn's disease in 1 case, respectively. Endoscopic or radiologic intervention for the dilatation of narrowed bile ducts were performed in 14.3% and 71.4%, respectively. Two cases (7.1%) underwent liver transplantation. CONCLUSION: Although PSC is a rare disease in Korea, we prospect that more cases will be reported as concern and knowledge of this entity increase.

Endoscopic Minor Papilla Intervention in a Patient with Traumatic Ductal Leak without Pancreas Divisum / 대한소화기내시경학회지

Endoscopic treatment has been performed in a variety of pancreatic ductal diseases such as stones, strictures, sphincter stenosis, and ductal disruption and is known to be an effective therapy in some patients. Endoscopic treatment through the minor papilla is frequently done in patients with pancreas divisum. Few data are, however, available concerning endoscopic minor papilla interventions in patients without pancreas divisum but with difficult access to the main pancreatic duct at the major duodenal papilla. We present a 49-year-old man, who had pancreatic ductal leak caused by previous pancreatic surgery. He was treated by endoscopic naso-pancreatic drainage and pancreatic ductal stenting through the minor papilla, so called pancreatic rendezvous technique. Follow-up ductography after two-weeks of naso-pancreatic drainage showed no leak. This case shows that minor papillar orifice could be an alternative access for therapeutic endoscopic intervention in a patient with traumatic pancreatic ductal leak without pancreas divisum.

Acute pericarditis caused by penetrated polymethylmethacrylate of the right ventricle after percutaneous vertebroplasty / 대한내과학회지

Percutaneous vertebroplasty (PVP) has been known as minimally invasive procedure to treat aggressive vertebral hemangioma, painful osteolytic vertebral tumors, and osteoporotic compression fractures. Some cases were reported to be associated with infrequent but serious complications of this procedure, such as severe arterial hypotension, pulmonary embolism, cerebral embolism, paraplegia, and bronchospasm. We report a case of acute pericarditis after PVP, which was treated successfully with open heart surgery.

Chronobiological Patterns of Acute Aortic Syndrome : Comparison with Those of Acute Myocardial Infarction

BACKGROUND AND OBJECTIVES: Chronobiological rhythms have been shown to influence the occurrence of a variety of cardiovascular disorders, including acute myocardial infarction (AMI). The present study investigated whether the onset of acute aortic syndrome (AAS) has unique chronobiological rhythms in Korean populations. SUBJECTS AND METHODS: The clinical data of 371 consecutive AAS patients, admitted between 1993 and 2003, were retrospectively analyzed; 310 AMI patients, who underwent primary percutaneous angioplasty in the hyperacute phase between 1998 and 2001, were also selected. RESULTS: In the AAS group, the final diagnoses were aortic dissection (AD) and aortic intramural hematoma (AIH) in 212 and 159 patients, respectively Similar to AMI, AAS showed a significantly higher occurrence from 6 AM to noon compared with other time periods (p=0.0013). AAS showed a second peak occurrence from 6 PM to midnight, which was not observed in the AMI group. A subgroup analysis revealed that younger patients (age < 60 years) and those with a past medical history of hypertension had the highest occurrence from 6 PM to midnight, which was quite different compared to the AAS patients. No significant variation was found for the day of the week in either group. Although no significant seasonal variation was observed in the frequency of AMI, the frequency of AAS was significantly higher during winter (p<0.001). The circadian and seasonal variations in the frequency of AIH were similar to those of AD. CONCLUSION: AAS shows unique circadian and seasonal variations in Korean populations. Our findings may have implications for the prevention of AAS by tailoring treatment strategies to ensure maximal benefits during the vulnerable periods.

A Clinical Study of the Rhegmatogenous Retinal Detachment

PURPOSE: This study aimed to analyze the clinical characteristics, results of operational method and causes of operational failure in rhegmatogenous retinal detachment except macula hole induced retinal detachment, and to analyze several factors of vision. METHOD: We reviewed the records of 720 patients, 743 eyes with regmatogenous retinal detachment who had undergone operation from January 1990 to December 1999, and followed up for at least 3 months or longer. RESULT: Scleral buckling (728 eyes, 98.0%), gas injection (9 eyes, 1.2%) and vitrctomy (6 eyes, 0.8%) were done as a primary operation. Anatomical success rate was 684 eyes(34.9%). A good visual acuity ( V A >or=0.5) was achieved in 239 eyes (34.9%), reasonable vision (VA 0.4~0.15) in 192 eyes(28.1%), ambulatory vision (VA 0.1~0.02) in 95 eyes (13.8%), whereas 158 eyes (23.2%) became blind (VA