We report two cases of malignant schwannoma of skin in association with non-familial neurofibromatosis. Case 1, a 47 year old man, had a large subcutaneous tumor on the sacral area and case 2, a 62 year old woman, a painful, ulcerating tumor on the posterior aspect of the left arm. Both cases were histopathologically confirmed as malignant schwannomas and immunohistochemical studies showed 5-100 protein in the tumor cells. After surgical excision of the tumors, case 1 was lost to follow up, while case 2 remained without evidence of disease for more than one and half years.
Arm ; Female ; Humans ; Lost to Follow-Up ; Neurilemmoma* ; Neurofibromatoses* ; Skin ; Ulcer

PURPOSE: To report the change of pupil sizes according to age and illuminance in the normal Korean population. METHODS: Normal Koreans outpatients who never had a history of ophthalmic disease were examined. The patients consisted of 320 eyes of 160 patients, which were classified into 8 age decades (teenage to 80's) with 40 eyes in each age group. The vertical and horizontal pupil size and area under 4 different illuminances (3,500, 1,200, 500, 5 lux) were measured using the Colvard pupillometer(R) (OASIS Medical, Glendora, CA, USA). RESULTS: The pupil size was significantly decreased as the age increased under each illuminance. The pupil area measured after dark adaptation was 47.30 mm2 in the teenage group, 43.32 mm2 in the 20's group, 41.94 mm2 in the 30's group, 40.98 mm2 in the 40's group, 40.61 mm2 in the 50's group, 38.60 mm2 in the 60's group, 37.78 mm2 in the 70's group and 35.45 mm2 in the 80's group. The decrease in pupil area was statistically significant. CONCLUSIONS: In the normal Korean population, a significant decrease in pupil size and area was observed with aging. The present study results provide good basic data for cataract and presbyopia refractive surgery.
Aging ; Cataract ; Dark Adaptation ; Eye ; Humans ; Outpatients ; Presbyopia ; Pupil ; Refractive Surgical Procedures

PURPOSE: To report a case of bacterial keratitis caused by Hafnia alvei after using a contact lens in a patient with graft-versus-host disease. CASE SUMMARY: A 47-year-old male patient presented with a corneal ulcer. The patient had been wearing a therapeutic contact lens for a month because of severe dry eye syndrome and filament removal due to filamentary keratitis. The patient had been taking moxifloxacin to prevent infection, but had not for the previous 7 days. The corneal ulcer occurred at the 11 o'clock direction in the peripheral cornea accompanied by corneal epithelial defect and stromal infiltration. The corneal scraping and culture yielded Hafnia alvei, which is susceptible to ceftazidime, levofloxacin and moxifloxacin. There was no systemic signs or symptoms to suspect systemic infection. After treatment with a topical antibiotic agent, the patient's eye condition improved with only mild corneal opacity remaining. CONCLUSIONS: Hafnia alvei infection rarely occurs in humans but should be considered as a potential causal pathogen in immunosuppressed patients.
Aza Compounds ; Ceftazidime ; Cornea ; Corneal Opacity ; Corneal Ulcer ; Dry Eye Syndromes ; Eye ; Graft vs Host Disease ; Hafnia ; Hafnia alvei ; Humans ; Keratitis ; Male ; Middle Aged ; Ofloxacin ; Quinolines

BACKGROUND: We aimed to evaluate the feasibility of using the allele burden of Janus kinase 2 (JAK2) V617F as a criterion for discriminating 3 subtypes of Philadelphia chromosome-negative myeloproliferative neoplasm (Ph-MPN): polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). METHODS: We collected 70 peripheral blood (PB) and 81 bone marrow (BM) samples from patients diagnosed with Ph-MPN. Real-time quantitative PCR (RQ-PCR) and Amplification Refractory Mutation System (ARMS) assays were performed for each sample. We compared the allele burden of JAK2 V617F for each subtype of Ph-MPN and determined the concordance rates of the results between the 2 tests. RESULTS: The JAK2 V617F allele burden differed significantly among the 3 disease categories in both PB (P=0.045) and BM (P=0.011) samples. Subsequent subgroup analysis revealed that the median allele burden of JAK2 V617F for ET (21.71% for PB and 24.95% for BM) was significantly lower than that for PV (56.88% for PB, P=0.047; 72.66% for BM, P=0.003) and PMF (56.16% for PB, P=0.050; 59.04% for BM, P=0.049). Concordance rate between the RQ-PCR and ARMS data was 90.7%. Of the 14 discrepant cases, 12 were RQ-PCR(+)/ARMS(-) and 2 were RQ-PCR(-)/ARMS(+). CONCLUSION: The allele burden of JAK2 V617F was significantly lower for ET than that for PV or PMF in both PB and BM samples. The JAK2 V617F allele burden is a diagnostic tool for differentiating PV or PMF from ET.
Alleles ; Arm ; Bone Marrow ; Diagnosis, Differential ; Discrimination (Psychology) ; Humans ; Janus Kinase 2 ; Myeloproliferative Disorders ; Philadelphia ; Polycythemia Vera ; Polymerase Chain Reaction ; Primary Myelofibrosis ; Real-Time Polymerase Chain Reaction ; Thrombocythemia, Essential

BACKGROUND: Therapy-related AML (t-AML) occurs as a late complication of chemotherapy administered to treat a prior disorder. Prognostic factors affecting the clinical outcome in t-AML have not yet been clearly defined; therefore, we evaluated these factors in this study. METHODS: Forty-eight patients diagnosed with t-AML within the past 10 years were enrolled, and their chemotherapy regimens categorized into 4 groups: alkylating agents (AK) only, topoisomerase II inhibitors (TI) and AK, TI only, and others. The prognostic factors affecting clinical outcome were evaluated. RESULTS: Five (10.4%), 21 (43.8%), 9 (18.8%), and 13 (27.0%) patients were treated with AK only, AK and TI, TI only, and others, respectively. Patients with an AML M3 phenotype showed significantly longer overall survival (OS; 55.1 vs. 14.3 months, P=0.040) and disease-free survival (DFS; 61.2 vs. 17.5 months, P=0.049) than other phenotypes. In contrast, patients with a complex karyotype showed significantly shorter OS (7.9 vs. 31.3 months, P=0.008) and DFS (9.5 vs. 38.6 months, P=0.046); additionally, patients with chromosome 5 or 7 abnormalities showed significantly shorter OS (9.1 vs. 30.7 months, P=0.011) than other phenotypes. Only the presence of a complex karyotype or AML M3 phenotype retained prognostic impact in a multivariate analysis. CONCLUSION: Only the AML M3 phenotype was identified as having a good prognosis, and this might suggest that it exhibits unique clinical features in t-AML patients. Moreover, our findings indicated that karyotype was the strongest prognostic indicator and predicted a poor prognosis for t-AML patients with a complex karyotype.
Alkylating Agents ; Chromosomes, Human, Pair 5 ; Disease-Free Survival ; Humans ; Karyotype ; Leukemia, Myeloid, Acute ; Phenotype ; Prognosis ; Topoisomerase II Inhibitors

No abstract available.
Dendritic Cells*

PURPOSE: To investigate the incidence and clinical characteristics of juvenile idiopathic arthritis (JIA)-associated uveitis in Korea and to identify the risk factors for developing uveitis and poor visual outcome. METHODS: We performed a retrospective chart review of 149 patients who were diagnosed as JIA between January 2001 and December 2012. Patients were classified based on the International League of Associations for Rheumatology (ILAR) criteria and the incidence and clinical course of uveitis according to each subtype was investigated. We also evaluated the risk factors for poor prognostic outcomes. RESULTS: The present study included 79 males and 70 females and the mean age was 7.42 +/- 3.82 years. In 13 patients (8.6%), uveitis manifested evenly among JIA subtypes, including 5 patients with oligoarthritis type, 4 patients with polyarthritis rheumatoid negative type, and 4 patients with systemic type. Anti-nuclear antibody (ANA) was detected more in the uveitic group (46.13%). The characteristics of uveitis were acute (85%), bilateral (85%), and anterior uveitis (92%). Chronic uveitis occurred in only 2 patients who had uveitis at an early age (mean age of 5 years) and had severe anterior chamber reaction at presentation. Poor visual outcome was associated with band keratopathy and posterior synechiae preceded by the chronic uveitis. CONCLUSIONS: JIA-associated uveitis occurred evenly in each subtype. Uveitis was increased in the presence of ANA. Most uveitis showed good visual outcomes but in cases of early uveitis onset and with severe anterior chamber reaction at presentation the risk of chronic uveitis increased resulting in poor visual outcomes.
Anterior Chamber ; Arthritis ; Arthritis, Juvenile Rheumatoid* ; Female ; Humans ; Incidence ; Korea* ; Male ; Retrospective Studies ; Rheumatology ; Risk Factors ; Uveitis* ; Uveitis, Anterior

The three-dimensional (3-D) shape of erythrocytes is strongly associated with various diseases. However, conventional optical imaging approaches with Wright's staining only provide information on two-dimensional morphology. Here, we employed optical diffraction tomography (ODT), a label-free 3-D quantitative phase imaging technique, and observed uniquely shaped red blood cells (RBCs) in the peripheral blood of a patient diagnosed with myelodysplastic syndrome. Peripheral blood samples were collected when the patient visited our hospital for his two out-patient follow-ups in May 2018. The 3-D tomograms of randomly chosen RBCs were reconstructed using a commercial ODT setup. From the reconstructed 3-D RBCs, 37.5% and 32.8% of RBCs demonstrated cup-like shapes at the first and the second out-patient follow-up, respectively. Even though this is a single case report, the finding is novel and can be a potential dyserythropoietic feature found in peripheral blood.
Erythrocytes ; Follow-Up Studies ; Humans ; Myelodysplastic Syndromes ; Optical Imaging ; Outpatients ; Refractometry

Anticoagulants ; Fibrinogen ; Heparin ; Heparin, Low-Molecular-Weight ; Plasma ; Reference Values

Lymphangioma of the large bowel is a rare benign tumor which is composed of numerous small or large thin-walled lymphatie spaces contained fibrous tissue, smooth muscle and aggregates of lymphoid tissue. Lymphangioma never demonstrated any potential for malignant degeneration. There are three types; l. simple capillary lymphangioma, 2 cavernous lymphangioma and 3 cystic lymphangioma. Endoscopically, lymphangioma appears as a compressible smooth, round, or oval submucosal tumor covered with pale intact mucosa. The surfaces is smooth and the superficial color is the same as the surrouding mucosa or slightly more yellowish. There are change of dimension and shape during propagation of the peristaltic wave and on compression. Recently, authors experienced 4 cases of lymphangioma of large intestine which has been revealed by typical colonoscopic findings and/or endoscopic ultrasound, and confirmed by surgical resection and colonscopic snare polypectomy. 3 of 4 cases show cystic lymphangioma and one case shows carvenous lymphangio~rna. We report these cases with review of literatures.
Capillaries ; Colon* ; Intestine, Large ; Lymphangioma* ; Lymphangioma, Cystic ; Lymphoid Tissue ; Mucous Membrane ; Muscle, Smooth ; SNARE Proteins ; Ultrasonography