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Purpose: The risk of opioid-related aberrant behavior (OAB) in Korean cancer patients has not been previously evaluated. The purpose of this study was to investigate the Opioid Risk Tool (ORT) in Korean cancer patients receiving opioid treatment.Methods: Data were obtained from a multicenter, cross-sectional, nationwide observational study regarding breakthrough cancer pain. The study was conducted in 33 South Korean institutions from March 2016 to December 2017. Patients were eligible if they had cancer-related pain within the past 7 days, which was treated with strong opioids in the previous 7 days.Results: We analyzed ORT results of 947 patients. Only one patient in each sex (0.2%) was classified as high risk for OAB. In patients with an ORT score of 1 or higher, the score primarily represented positive responses for personal history of depression, personal or family history of alcohol abuse, or 16–45 years age range. These patients had more severe average and worst pain scores and used rescue analgesics more frequently than patients with ORT scores of 0. The proportion of moderate- or high-risk patients according to ORT was lower in patients receiving low doses of long-acting opioids than in those receiving high doses (2.0% vs. 6.6%, p = 0.030). Moderate or high risk was more frequent when ORT was completed in an isolated room than in an open, busy place (2.7% vs. 0.6%, p = 0.091).Conclusion: The risk of OAB measured by ORT was very low in cancer patients receiving strong opioids for analgesia.

Migraine patients often complain of repeated attacks of monocular visual disturbance including scintillations, scotomas, blindness and pupillary dysfunction. Temporary pupillary enlargement of the ipsilateral side during migraine attack, although rarely reported, is referred to as an Adie’s-like tonic pupil [1]. Adie’s tonic pupil is caused by denervation of the postganglionic parasympathetic pupillary nerves, with light-near dissociation, and cholinergic supersensitivity. Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS) is characterized by early-onset spastic ataxia, peripheral sensorimotor neuropathy, and pyramidal symptoms. ARSACS is caused by mutations in the SACS gene, which encodes the Sacsin expressed in various tissues including brain motor systems. Sacsin is believed to integrate the ubiquitin-proteasome system and Hsp70 chaperone machinery, which are important components of the cellular response associated with neurodegenerative diseases [2]. Here, we describe a patient suffering from chronic migraine without aura who developed cerebellar ataxia and unilateral mydriasis during acute migraine persisted after resolved headache. Presence of a SACS gene mutation is an expansion of the clinical phenotype associated with SACS mutations to ciliary ganglioplegic migraine. A 47-year-old female who suffers from long standing headache developed unsteady gait and poor coordination. With a diagnosis of migraine without aura based on the third edition of the International Classification of Headache Disorders (ICHD-III) criteria, she was prescribed preventive medicine with topiramate and onabotulinumtoxinA injection on occasion. Physical examination revealed a dilated pupil in the left eye without reflexive constriction to light or accommodation which persisted after resolution of migraine. Except for anisocoria, no other abnormal findings were identified. Remarkable reduction of the left pupil size was observed in response to 0.125% pilocarpine (Figure 1). The patient also showed mild nonataxic spastic paraplegia but without distal weakness or pyramidal signs. A family history of migraine headache and other neurological disorders was denied. Nerve conduction studies confirmed the mixed demyelinating and axonal character of polyneuropathy. Brain MRI with angiography did not reveal any abnormalities.

Aims To investigate the influence of preterm birth on the optic disc and retinal vessels by measurements of cup-to-disc (C/D) ratio and arteriole-to-venule (A/V) ratio. Methods Eighty-three eyes of 42 preterm births were included. In the age- and sex-matched control group, 83 eyes of 42 full-term births were used. Fundus color photographs were taken. ImageJ software was used to calculate C/D and A/V ratios from the fundus images. Results Fundus photographs were taken at 8.01 ± 2.22 years of age for the preterm group and 8.01 ± 2.13 years of age for the control group. The mean gestational age of the preterm group was 27(4)/7 weeks (range, 24-34 weeks). The preterm group had significantly larger C/D ratio and smaller A/V ratio (mean ± standard deviation: 0.46 ± 0.12 and 0.59 ± 0.08, respectively) than the control group (0.36 ± 0.07 and 0.68 ± 0.07, respectively) after spherical equivalent refractive error was adjusted. Conclusions Preterm birth is significantly associated with larger C/D ratio and smaller A/V ratio. These findings show the effect of preterm birth on the development of optic disc and retinal vessel development.

Background: Head and neck squamous cell carcinomas (HNSCCs) display cellular heterogeneity and contain cancer stem cells (CSCs). Sex-determining region Y [SRY]-box (SOX)2 is an important regulator of embryonic stem cell fate and is aberrantly expressed in several types of human tumours. Nonetheless, the role of SOX2 in HNSCC remains unclear. Methods: We created cells ectopically expressing SOX2 from previously established HNSCC cells and examined the cell proliferation, self-renewal capacity, and chemoresistance of these cells compared with control cells. In addition, we knocked down SOX2 in primary spheres obtained from HNSCC tumour tissue and assessed the attenuation of stemness-associated traits in these cells in vitro and in vivo. Furthermore, we examined the clinical relevance of SOX2 expression in HNSCC patients. Results: SOX2 is aberrantly expressed in primary tissue of HNSCC patients but not in healthy tissue. SOX2 expression correlated with tumour recurrence and poor prognosis of HNSCC patients. Ectopic expression of SOX2 induced cell proliferation via cyclin B1 expression and stemness-associated features, such as self-renewal and chemoresistance. In addition, a knockdown of SOX2 in HNSCC CSCs attenuated their self-renewal capacity, chemoresistance (through ABCG2 suppression), invasion capacity (via snail downregulation), and in vivo tumorigenicity. Conclusions: These results suggest that SOX2 may have important roles in the ‘stemness' and progression of HNSCC. Targeting SOX2-positive tumour cells (CSCs) could be a new therapeutic strategy in HNSCCs.

BACKGROUND Extraocular muscle (EOM) injury is a rare but serious complication of endoscopic sinus surgery (ESS). The aim of this study is to describe the clinical characteristics and course of EOM injury occurring during ESS. DESIGN Retrospective case series. METHODS Medical records and CT images of patients who suffered from EOM injury after ESS between 2006 and 2012 were retrospectively reviewed. Patient demographics, endoscopic anatomy, type of surgery (primary or revision), predisposing risk factors, site and extent of injury on CT imaging, and associated complications were evaluated. In addition, data regarding ophthalmologic management and clinical outcomes were collected. RESULTS Ten patients with EOM injuries after ESS were included in this study. One patient was undergoing revision ESS. All patients sustained medial rectus muscle injury and one patient suffered concurrent ipsilateral inferior rectus muscle injury. A microdebrider was used in nine cases. Right-sided injury (90% of patients) was more prevalent than left-sided injury, and 70% of injured medial rectus muscles were completely transected. After subsequent strabismus surgery, 8/9 patients regained binocular single vision in primary gaze despite residual diplopia in some gaze positions. CONCLUSION Although proper ophthalmologic surgery after EOM injury may improve deviation in the primary gaze position, none of the patients regained normal EOM movement. Therefore, prevention of this complication through adequate surgical technique and precautions is important.

Impaired neurogenesis has been associated with brain disorders. The role of peroxiredoxin 6 (PRDX6) in the neurodegenerative diseases is very controversial. To demonstrate the role of PRDX6 in neurogenesis, we compared neurogenesis ability and studied the molecular mechanisms. It was found that the neurogenesis of neural stem cells and expression of the marker protein were lowered in PRDX6 Tg-mice compared with non-tg mice. Moreover, the expression of wdfy1 was dramatically decreased in PRDX6-Tg mice, also, we observed that wdfy1 siRNA decreases the differentiation ability of primary neural stem cells to astrocyte and neuronal cells as well as PC12 cells. However, knockdown of PRDX6 recovered neurogenesis in the brain of PRDX6-Tg mice as well as PC-12 cells. We also showed that TLR4 was dramatically reduced in PRDX6 Tg mice as well as PC-12 cells and PRDX6 overexpression reduced neurogenesis was rescued after treatment of TLR4 siRNA. We further found that reduced TLR4 expression and neurogenesis was reversed in the neuron from PRDX6-Tg mice as well as PC12 cells by introduction of wdfy1 plasmid. Moreover, TLR4 siRNA reduced neurogenesis and wdfy1 expression. This study indicated that PRDX6 inhibits neurogenesis of neural precursor cells through TLR4 dependent downregulation of wdfy1.

AimsThe aims of this study were to investigate the longitudinal change in lateral rectus (LR) muscle insertion after recession surgery, and to evaluate a relationship between insertion distance and postoperative amount of deviation.MethodsWe recruited 31 patients who underwent primary LR recession surgery with normal anterior segment structures. An AS-OCT scan of the LR muscle was performed at every visit. Data on sex, age, degree of deviation (prism diopter), and spur-LR insertion distance using AS-OCT were collected at preoperatively and postoperative months 1, 3, and 6. Spur-LR insertion was defined as the shortest distance between the insertion of the LR muscle and the scleral spur was measured using the caliper function in the AS-OCT software.ResultsThe mean distance of spur-LR insertion measured with AS-OCT was 5.5±0.7 mm preoperatively, and 11.1±0.9 mm at 1 month, 11.5±0.8 mm at 3 months, and 11.0±0.7 mm at 6 months postoperatively. There were significant differences in spur-LR insertion between postoperative 1 and 3 months, and between 3 and 6 months (generalized estimating equation, all P0.001). The overall spur-LR insertion measurement has a significantly negative correlation with postoperative amount of deviation (Pearson's correlation, P=0.035, r=-0.218).ConclusionsAS-OCT is a useful instrument for understanding postoperative changes in EOM after tissue swelling subsides.

Pancreatic cysts are becoming increasingly recognized in clinical practice. Despite our improved awareness and understanding of these lesions, making a correct diagnosis and determining their malignant potential remains challenging. We aim to provide an overview of pancreatic cysts including classification, diagnosis, and recommendations for treatment and surveillance in recent consensus guidelines.

We investigated outcomes according to a new clinical grading system for chronic graft-versus-host disease (chronic GVHD) in 38 patients who developed chronic GVHD after an allogeneic hematopoietic stem cell transplantation. We categorized the patients into three grade groups, namely, grade I, grade II and grade III, according to the presence of three risk factors: extensive skin involvement, thrombocytopenia (TP) and progressive type of onset. Sixteen patients were classified into grade 1, 19 into grade II and three into grade III. The probability of withdrawal of systemic immunosuppression (IST) at 1, 2 and 3 years was 61, 76 and 87%, respectively. Patients with grades 2 or 3 chronic GVHD had prolonged duration of systemic IST compared to grade 1 (P=0.043). The probability of GVHD-specific survival (GSS) at 5 years was 52%. Twenty-two of 38 patients with chronic GVHD were still alive and the estimated 3-year overall survival (OS) rate was 60%, whereas that for the group with chronic GVHD grade I and grade II+III was 64 and 48% (P0.05). Multivariate analysis showed that prior occurrence of acute GVHD, chronic GVHD grade, serum bilirubin over 1.5 mg/dl, date of diagnosis of chronic GVHD (day 150 versusday 150) and transplantation-risk factor were independent prognostic factors for GSS and OS.

Background: This nonrandomized open label phase II study evaluated the efficacy and safety of FOLFOXIRI in metastatic or recurrent gastric cancer patients. Patients and methods: Patients with histologically proven, metastatic gastric adenocarcinoma, aged 18–70 years, performance status zero to two, no prior chemotherapy, and with signed written informed consent were eligible. Treatment consisted of irinotecan 150 mg/m2 day 1, oxaliplatin 85 mg/m2 day 1, leucovorin 100 mg/m2 day 1, and 5-fluorouracil 2000 mg/m2 as a 48-h continuous infusion starting on day 1, which was repeated every 2 weeks. Results: From August 2004 to August 2005, 48 patients were prospectively enrolled. The median age was 54 years (24–69). In total, 386 cycles were administered with a median of nine cycles per patient (range 1–12 cycles) and 45 of 48 patients were assessable for treatment response. An independent review of tumor responses resulted in overall response rate of 66.7% (95% confidence interval = 53.4% to 80.0%) by intent-to-treat analysis with one complete response and 31 partial responses. The median survival of all patients was 14.8 months and the median time to progression was 9.6 months. Most common grade 3/4 toxic effects were neutropenia (12% of all cycles) and emesis (8% of all cycles). Grade 2 peripheral neuropathy occurred in five patients. One (2%) patient had severe tumor bleeding and five (10%) patients experienced grade 3 diarrhea. Conclusions: The modified FOLFOXIRI combination chemotherapy showed a very promising preliminary antitumor activity and was generally well tolerated as a first-line treatment of patients with metastatic gastric cancer.

Nongastric marginal zone B-cell lymphoma (NG-MZL) is a relatively uncommon indolent lymphoma. From 1990 to 2005, a total of 247 patients with histologically confirmed NG-MZL were analyzed. Ann Arbor stage I/II disease was present in 78% (167 out of 215). One hundred eighty-six patients out of two hundred eight were categorized into the low/low-intermediate risk group (89%) according to International Prognostic Index (IPI). Eighty percent (172/215) were in low risk group according to Follicular Lymphoma International Prognostic Index (FLIPI). Complete and partial remissions (CR and PR) were achieved in 140 (92.7%) and 8 (5.3%) of the 151 stage I/II patients. Especially, radiation containing treatment achieved 96% CR rate (108 out of 113). In 38 patients with stage III/IV, CR and PR were achieved in 17 (44.7%) and 11 (26.3%), respectively. The estimated five-year overall survival (OS) and progression-free survival (PFS) were 93.8% and 70.1%, respectively. Although anthracycline-containing regimen could achieve higher CR rate, it did not improve PFS. Stage III/IV, low hemoglobin, poor performance status, high/high-intermediate IPI, poor risk FLIPI, and nodal MZL were poor prognostic factors for PFS. NG-MZL is an indolent disease. FLIPI has strong power to predict the prognosis of NG-MZL.

PurposeThe purpose of this study is to describe the clinical characteristics and treatment results of medial rectus muscle (MR) transection incurred during endoscopic sinus surgery.MethodsThis retrospective study included 16 patients with MR transection incurred during endoscopic sinus surgery between 1994 and 2015. The operative notes of the surgical procedure, the pattern of strabismus, the type of muscle injury, the type of corrective strabismus surgery, and the surgical outcomes were reviewed.ResultsNine patients had partial resection of MR and seven patients had complete transection of MR, resulting from an injury incurred during endoscopic sinus surgery. Three of the nine patients with partial resection injury were initially diagnosed as complete resection and subsequently re-diagnosed as partial resection in a review of the images during this study. Five of the nine patients with partial MR resection underwent only simple recession/resection surgery. Patients with complete MR transection underwent muscle transposition or globe fixation surgeries and often multiple operations were required.ConclusionsThe results of this study showed that the treatment strategies could vary depending on the nature of muscle injury. In cases with complete transection, muscle transposition or globe fixation surgeries are often required, with multiple operations. However, partial muscle resection with only simple recession/resection surgery shows a favorable outcome in many cases. The use of proper imaging techniques, a thorough review of the images with various planes, and close follow-up are important for determining the nature of the muscle injury.

The absence of focal choroidal excavation in children and adolescents without retinal or choroidal disorders or ocular trauma

Nodal marginal zone B-cell lymphoma (NMZL) is a relatively uncommon type of lymphoma. Because of the rarity, the natural history and the optimal treatment modality have not been well defined. Therefore, we performed a retrospective analysis of the clinical features and treatment outcomes of NMZL. Thirty-six patients who were histologically diagnosed as NMZL were included in the analysis. Fifty-three percent of the patients had localized disease (stages I and II), and 21.2% (7/33) had bone marrow involvement at presentation. B symptom was present in only three patients (8.3%). Most patients were categorized as low or low-intermediate risk group by international prognostic index (IPI) (77.1%). Majority (94.4%) of the patients with localized disease achieved complete remission (CR) after the initial treatment. Of the seven patients with disseminated disease, who were treated with anthracycline-based chemotherapy, four patients achieved CR. Of the seven patients who received nonanthracycline-based chemotherapy, no patient achieved CR. After the median follow-up duration of 36 months, the median progression-free survival (PFS) was 3.9 (95% CI; 2.9-5.6) years, and the estimated 5-year PFS and overall survival rates were 47.2 and 82.7%, respectively. The significant predictive factors for PFS were performance status, advanced stage, and follicular lymphoma IPI (FLIPI) in this study. This clinical feature is similar to FL rather than to MZL-MALT type.

Overproduction of nitric oxide via inducible nitric oxide synthase (iNOS) is suggested to be a significant pathogenic factor in Helicobacter pylori induced gastritis. The purpose of this study was to examine the role of iNOS in H pylori associated gastric carcinogenesis.Two types of mice were used in this study: iNOS deficient mice (iNOS-/-) and wild-type littermates. Gastric cancer was generated in mice using a combination treatment comprising N-methyl-N-nitrosourea administration and H pylori infection. Fifty weeks after treatment, tumours in gastric tissues from both types of mice were examined using histopathology, immunohistochemistry, and immunoblotting for iNOS and 3-nitrotyrosine.The overall incidence of gastric cancer at week 50 was significantly lower in iNOS-/- compared with iNOS wild-type mice (p0.05). When analysed according to tumour pathology, the incidence of gastric adenocarcinoma was significantly lower in iNOS-/- compared with iNOS wild-type mice (p0.05). Immunostaining for iNOS was clearly observed in adenocarcinoma cells of iNOS wild-type mice, and was characterised by a strong cytoplasmic expression pattern. 3-Nitrotyrosine was expressed mostly in the area of the lamina propria of gastritis and adenoma lesions in iNOS wild-type mice. Immunoblotting analyses showed that iNOS and 3-nitrotyrosine were also expressed in both adenoma and adenocarcinoma tissues from iNOS wild-type mice. iNOS and 3-nitrotyrosine expression was greater in tumour tissues than in non-tumour tissues.These findings suggest that iNOS contributes to H pylori associated gastric carcinogenesis in mice.

To compare retinal nerve fiber layer (RNFL) thickness profiles between preterm and full-term children and to investigate factors affecting the RNFL distribution in preterm children. We performed Spectral domain optical coherence tomography (SD-OCT) peripapillary RNFL circular scan centered on the optic disc in 50 premature and 58 full-term children. RNFL thickness profiles were compared between preterm and full-term children using a linear regression model. Among preterm patients in this study, 20 patients previously received laser treatment for severe retinopathy of prematurity (ROP). Global average, nasal, and superior disc RNFL thickness profiles were significantly smaller in preterm children (92.70±16.57 μm, 56.02±17.04 μm, and 108.74±27.36 μm, respectively) compared with full-term children (101.63±9.21 μm, P=0.006, 69.14±14.15 μm, P

The purpose of this study was to compare the surgical outcomes of intraocular lens (IOL) refixation with intraocular lens exchange using perfluorocarbon liquid (PFCL) and fibrin glue-assisted sutureless scleral fixation surgery in patients with dislocation of the IOL. Twenty-five eyes of 25 patients who underwent surgery for dislocated IOLs with PFCL and fibrin glue-assisted scleral fixation were studied; 13 eyes experienced IOL refixation (in-the-bag and out-of-the-bag), and 12 eyes experienced IOL exchange. Preoperative and postoperative clinical features from patient charts and 25 eyes with >6 months' follow-up information were reviewed and analyzed. At postoperative 6 months, best-corrected visual acuity (BCVA) and spherical equivalent of IOL refixation and exchange were significantly improved (P=0.042, P=0.001), and endothelial cell density was significantly decreased in the two groups with no significant difference between them. Surgically induced astigmatism of IOL refixation improved from 0.90±0.47 to 0.61±0.37 (P=0.012), and IOL exchange improved from 1.17±0.64 to 0.73±0.37 (P=0.037) at postoperative 6 months, with no significant difference between the two groups. Complications occurred in four eyes in the IOL refixation group and in three eyes in the IOL exchange group. PFCL and fibrin glue-assisted IOL sutureless scleral refixation or exchanged fixation was an effective surgical treatment for IOL dislocation. Also, because postoperative BCVA, surgical outcomes, and complications did not differ significantly between IOL refixation and exchange surgery, if IOL exchange surgery is not indicated, IOL refixation surgical techniques should be considered.

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Introduction Although the use of broad-spectrum antibiotics in women with preterm premature rupture of membranes (PPROM) is recommended to prolong pregnancy and decrease short-term neonatal complications, the optimal regimen remains undetermined. The objective of this study was to compare the efficacy of cefazolin plus macrolide (erythromycin or clarithromycin) versus cefazolin alone in reducing neonatal morbidity and placental inflammation for women with PPROM. Methods This prospective study included singleton pregnancies with PPROM (23–33 weeks gestation). The primary outcome was neonatal composite morbidity and the secondary outcomes were the incidence of abnormal brain sonography and infant neurological outcome at one year of age. The presence and the stage of acute histological chorioamnionitis and funisitis were also reviewed blinded to all clinical information. Results 102 women were randomly assigned to cefazolin ( n = 35), cefazolin plus erythromycin ( n = 31), or cefazolin plus clarithromycin ( n = 36). The neonatal composite morbidity, the incidence of abnormal brain sonography, and infant neurological outcome at one year of age were similar between the comparison treatments (combination of cefazolin plus erythromycin or clarithromycin) and cefazolin. However, the presence and stage of histological funisitis showed significant difference between cefazolin plus clarithromycin versus cefazolin alone ( p = 0.023). Discussion This study is the first clinical trial of the use of cefazolin with either clarithromycin or erythromycin compared to cefazolin alone in the management of PPROM in which the primary and secondary analyses showed no difference among the three antibiotic regimens. The only noted difference was from a lesser degree of histological funisitis associated with clarithromycin exposure. Conclusion Our data suggests that clarithromycin may be an alternative worth considering with potentially beneficial effects compared to erythromycin in PPROM.

Although external-beam radiation therapy (EBRT) has been an effective treatment modality in patients with bilateral advanced retinoblastoma, it significantly increases the risk of second malignancies and facial deformities. This study aimed to evaluate the efficacy of tandem high-dose chemotherapy and autologous stem cell rescue (HDCT/ASCR) for treatment, instead of EBRT, in children with bilateral advanced retinoblastoma. Fourteen patients with bilateral retinoblastoma received chemotherapy, and local therapy was provided whenever possible. When at least one functional eye could not be saved by chemoreduction and local therapy, tandem HDCT/ASCR was provided to avoid EBRT. As a result, nine patients received tandem HDCT/ASCR. The toxicities were tolerable and there was no TRM. All nine patients who received tandem HDCT/ASCR had at least one functional eye without EBRT, and in two patients, both eyes were saved. No second malignancy has developed to date. HDCT/ASCR might be an effective treatment for bilateral advanced retinoblastoma, especially in cases in which at least one functional eye could not be preserved with chemoreduction and local therapy alone, and where EBRT was unavoidable. Long-term follow-up and further studies are needed to evaluate the efficacy and toxicity of HDCT/ASCR as an alternative treatment to EBRT.

1 Antimony (Sb), an industrial and environmental toxicant, is known to cause dermatitis and pulmonary inflammations, but the immunomodulatory effects of environmental or occupational exposure to Sb have not been reported. To initiate investigation of Sbinduced alterations of the immune system, the concentrations of the IgG subclasses, IgE, interleukin-2, interferon-g, and interleukin-4 in sera obtained from workers exposed to Sb through antimony trioxide manufacture were determined and compared with those of control subjects. 2 The serum levels of IgG1, an immunoglobulin involved in host defense against many microbial infections, were significantly lower in the sera of Sb-exposed individuals than the controls. The serum concentrations of IgE, an immunoglobulin mediating allergic hypersensitivity, also were lower in the Sb-exposed workers than the controls. In addition, the levels of interleukin-2 and interferon-g, multifunctional cytokines for T-cell mediated immunity, were lowered in the factory workers. 3 A significant positive correlation between IgG4 and urine Sb levels was found among the Sb-exposed workers, indicating a possible role of IgG4 in Sbmediated pulmonary or skin pathogenesis. 4 The present study provides the first report on immune alterations induced by occupational exposure to Sb and suggests that Sb exposure disturbs immunohomeostasis in humans observed as a function of aberrant serum cytokine and immunoglobulin levels, which could influence health.

Background: Hepatic veno-occlusive disease (VOD) is a devastating regimen-related toxicity after hematopoietic stem cell transplantation (HSCT). There is no safe and proven therapy for established VOD, and focus has been on its prevention. Previous studies have shown that ATIII level is markedly decreased in patients with VOD. We conducted a prospective, randomized trial to compare heparin plus ATIII versus heparin alone in HSCT recipients. Methods: A clinical diagnosis of hepatic VOD was defined as satisfying two of the following criteria: bilirubin > 2.0 mg/dl, unexplained weight gain of > 2% from baseline, and hepatomegaly. The levels of PAI-1, TFG-β and TNF-α were measured at weekly basis for correlative analysis. Patients were randomized to receive or not receive prophylactic ATIII 1000U twice daily from day 1 until day +14 post-HSCT plus heparin 5U/kg/hr from day 1 until day +21 or discharge after HSCT. Results: Thirty patients were enrolled for the study. Fourteen patients received heparin + ATIII and 16 patients received heparin alone for VOD prophylaxis during HSCT. There were 9 AML, 6 NHL, 4 ALL, 3 CML, 2 ABL, 2 MM, 1 MDS, 1 HD, 1 AA and 1 SLL patient. Of the 30 patients enrolled, 13 patients received sibling allogeneic HSCT, 13 autologous, 3 unrelated allogeneic and 1 cord blood HSCT. VOD occurred in 4 patients (25%) in the heparin alone group and none in the heparin + ATIII group (0%, P = 0.044). The incidence of bleeding complication was higher in the heparin alone group (4/16, 25%) than the heparin + ATIII group (1/14, 7%) without statistical significance (P = 0.190). The mean baseline level of PAI-1 was slightly higher in the heparin + ATIII group (30.9 ± 31.0 ng/ml) when compared to the heparin group (18.9 ± 13.0 ng/ml, P = 0.062). The PAI-1 level decreased markedly in the heparin + ATIII group (23.0 ±10.1 ng/ml) by the third week following VOD prophylaxis therapy (heparin group; PAI level 40.3 ± 40 ng/ml, P = 0.010). The mean baseline levels of TGF-β and TNF-α did not differ significantly between the two groups. There was no VOD-related death. Conclusions: Although it was a preliminary analysis, the combination of ATIII and heparin for VOD prophylaxis in HSCT recipients seems to be more effective than heparin alone without increasing the hemorrhagic complication.

Background: Due to the rarity of the disease, prospective clinical trials on optimal treatment of intestinal non-Hodgkin’s lymphoma have been scarcely performed. Methods: Patients with; age > 18 years, pathologically proven diffuse large B cell lymphoma (DLBL), normal bone marrow, liver, renal and cardiac function were eligible. Patients with Burkitt’s lymphoma, lymphoblastic lymphoma, mantle-cell lymphoma, or infection with human immunodeficiency virus were excluded. All patients were staged after surgery according to the Ann Arbor classification modified by Musshoff et al. and Paris staging system (TNM system). Postoperative chemotherapy with CHOP regimen was administered for 6 cycles. CHOP consisted of cyclophosphamide 750 mg/m2 d1, doxorubicin 50 mg/m2 d1, vincristine 1.4 mg/m2 (max 2.0 mg/m2) d1, and prednisolone 100mg d1–5 repeated every 3 weeks. Results: From 1999 to 2004, 41 patients were enrolled. The median age was 50 years (range, 26 – 84) and male:female ratio was 29:12. All patients had clinical stage I/IIE DLBL and underwent surgical resection. Of the 41 patients, 3 patients refused postoperative CHOP chemotherapy. The 5-year DFS and OS were 83.3% and 89.1%, respectively. Surgicopathologic staging of all patients according to the Ann Arbor classification revealed 34 patients with stage I, 11 patients stage II1, 1 patent stage II2 and 3 patients with stage IIE. The Ann Arbor stage significantly predicted the survival of intestinal DLBL (p = < 0.0001). The T staging according to Paris staging system did not correlate with survival (p = 0.1456) while the N staging showed significant predictability (p = Conclusion: Surgical resection followed by 6 cycles of CHOP chemotherapy was a highly effective treatment scheme for localized intestinal DLBL patients. The Ann Arbor system seems to predict survival better than the TNM system.

Purpose: Nongastric marginal zone B-cell lymphoma (NG-MZL) is a relatively uncommon indolent lymphoma. We report a retrospective analysis of 247 patients with .NG-MZL, presenting their clinical features and therapeutic outcomes. Methods: From 1990 to 2005, a total of 247 patients with histologically confirmed NG-MZL were analyzed. Results: The median age was 49 years (range, 13–89 years). The study involved 129 males (52.2%) and 118 females (47.8%) The most common involving site was orbit and ocular adnexa (48.6%) followed by lymph node and lymphatic organs (17.8%), bowel (9.3%), lung (6.1%), thyroid gland (4.9%), salivary gland (4.5%) in the decreasing order of frequency. Ann Abor stage I/II disease was present in 78%(167 out of 215). BM involvement was less than 10%(19 out of 211). B symptom was observed in only 2%. One and eighty-six patients out of 208 were in low or low-intermediate risk group (89%) according to international prognostic index(IPI). Eighty percents (172/215) were in low risk group in follicular lymphoma international prognostic index (FLIPI). Patients were treated with a variety of therapeutic strategies. Complete and partial remissions were achieved in 139(92%) and 8(5.3%) of the 151 stage I/II patients, respectively, with an overall response rate of 97.3%. Especially, radiation containing treatment achieved 96% CR rate (108 out of 113). In 38 patients with stage III/IV, CR and PR were achieved in 17(44.7%) and 11(28.9%). The estimated 5-year overall survival (OS) and progression free survival (PFS) were 93.8% and 70.1%, respectively. Although anthracyclin contaning regimen could achieve higher CR rate, it did not improve PFS. Stage III/IV, low hemoglobin, high/high-intermediate IPI, poor risk FLIPI and nodal MZL were poor prognostic factors for PFS in multivariate analysis. Conclusion: NG-MZL is an indolent disease. For localized disease radiation achieved excellent local control. Anthracyclin containing chemotherapy cannot improve outcome. Both IPI and FLIPI can be applied to predict the prognosis.