BACKGROUND: JL1, a CD43 epitope and mucin family cell surface glycoprotein, is expressed on leukemic cells. An anti-JL1 antibody combined with a toxic substance can have targeted therapeutic effects against JL1-positive leukemia; however, JL1 expression on bone marrow (BM) lymphoma cells has not been assessed using flow cytometry. We investigated JL1 expression on BM lymphoma cells from patients with non-Hodgkin lymphoma (NHL) to assess the potential of JL1 as a therapeutic target. METHODS: Patients with BM involvement of mature B-cell (N=44) or T- and natural killer (NK)-cell (N=4) lymphomas were enrolled from May 2015 to September 2016. JL1 expression on BM lymphoma cells was investigated using flow cytometry. Clinical, pathological, and cytogenetic characteristics, and treatment responses were compared according to JL1 expression status. RESULTS: Of the patients with NHL and BM involvement, 37.5% (18/48) were JL1-positive. Among mature B-cell lymphomas, 100%, 38.9%, 33.3%, 100%, and 25.0% of Burkitt lymphomas, diffuse large B-cell leukemias, mantle cell leukemias, Waldenstrom macroglobulinemia, and other B-cell lymphomas, respectively, were JL1-positive. Three mature T- and NK-cell NHLs were JL1-positive. JL1 expression was associated with age (P=0.045), complete response (P=0.004), and BM involvement at follow-up (P=0.017), but not with sex, performance status, the B symptoms, packed marrow pattern, cytogenetic abnormalities, or survival. CONCLUSIONS JL1 positivity was associated with superior complete response and less BM involvement in NHL following chemotherapy.

BACKGROUND/AIMS: Stress has a role in the pathogenesis of functional dyspepsia (FD) and influences food intake in humans and animals. Prokinetic drugs have been used in FD, and some of these drugs reverse the feeding inhibition (FI) induced by acute restraint stress in rats. We aimed to evaluate the effect of DA-9701 on FI induced by acute restraint in rats. MATERIALS AND METHODS: Male Sprague-Dawley rats were divided into 6 groups: Control (no stress), Stress+vehicle, and Stress+DA-9701 at doses of 1, 3, 10, and 30 mg/kg (n=6~7). DA-9701 or vehicle was administered through gastric gavage 45 minutes before stress. After 60 minutes of stress, pre-weighed chow was given and the weight of remaining food was measured 30 and 60 minutes later. The effect of DA-9701 on FI was compared after pretreatment with WAY100635, a 5HT1A antagonist. RESULTS: The restraint stress group had significantly less food intake than the control group. After feeding, rats given 1 and 3 mg/kg of DA-9701 showed increased food intake at 60 minutes, but this was not statistically significant. Rats given 10 mg/kg of DA-9701 showed significantly increased food intake at 30 minutes and 60 minutes (P < 0.05). Interestingly, rats given 30 mg/kg of DA-9701 showed a significant decrease in food intake, similar to that of the vehicle group. The beneficial effect of 10 mg/kg of DA-9701 on FI was abolished by the pretreatment with WAY100635. CONCLUSIONS: Acute restraint stress reduced food intake in rats and pretreatment with DA-9701 improved stress-induced FI.
Animals ; Dyspepsia ; Eating ; Humans ; Male ; Rats ; Rats, Sprague-Dawley ; Stress, Physiological

BACKGROUND: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Alveolar RMS (ARMS) is characterized by FOXO1-related chromosomal translocations that result in a poorer clinical outcome compared with embryonal RMS (ERMS). Because the chromosomal features of RMS have not been comprehensively defined, we analyzed the clinical and laboratory data of childhood RMS patients and determined the clinical significance of chromosomal abnormalities in the bone marrow. METHODS: Fifty-one Korean patients with RMS < 18 years of age treated between 2001 and 2015 were enrolled in this study. Clinical factors, bone marrow and cytogenetic results, and overall survival (OS) were analyzed. RESULTS: In total, 36 patients (70.6%) had ERMS and 15 (29.4%) had ARMS; 80% of the ARMS patients had stage IV disease. The incidences of bone and bone marrow metastases were 21.6% and 19.6%, respectively, and these results were higher than previously reported results. Of the 40 patients who underwent bone marrow cytogenetic investigation, five patients had chromosomal abnormalities associated with the 13q14 rearrangement. Patients with a chromosomal abnormality (15 vs 61 months, P=0.037) and bone marrow involvement (17 vs 61 months, P=0.033) had a significantly shorter median OS than those without such characteristics. Two novel rearrangements associated with the 13q14 locus were detected. One patient with concomitant MYCN amplification and PAX3/FOXO1 fusion showed an aggressive clinical course. CONCLUSIONS: A comprehensive approach involving conventional cytogenetics and FOXO1 FISH of the bone marrow is needed to assess high-risk ARMS patients and identify novel cytogenetic findings.
Arm ; Bone Marrow* ; Child* ; Chromosome Aberrations ; Cytogenetics* ; Humans ; Incidence ; Neoplasm Metastasis ; Rhabdomyosarcoma* ; Sarcoma ; Translocation, Genetic

Precursor B-cell acute lymphoblastic leukemia (ALL), which is the most common subtype of pediatric acute leukemia, generally has a good prognosis. However, the prognosis also depends on the genetic abnormalities of the leukemic blast. Concurrent MYC and IGH/BCL2 translocations have recently been reported as a “double hit” in adult patients, but non-immunoglobulin (non-IG)/MYC translocation has rarely been reported. In this paper, we report a case of pediatric precursor B-cell ALL associated with translocations (14;18)(q32;q21) and (8;9)(q24;p13). The patient was a previously healthy 13-year-old boy. Complete remission was not achieved after first-line four-drug induction chemotherapy; thus, intensive salvage regimen, including high-dose cytarabine and L-asparaginase, were administered, which resulted in morphologic remission. However, his disease relapsed during the second cycle of salvage regimen, and he died of sepsis-induced multiorgan failure.
Adolescent ; Adult ; Cytarabine ; Humans ; Induction Chemotherapy ; Leukemia ; Male ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Precursor Cells, B-Lymphoid ; Prognosis

OBJECTIVE: Described herein is a microcatheter looping technique to facilitate aneurysm selection in paraclinoid aneurysms, which remains to be technically challenging due to the inherent complexity of regional anatomy. MATERIALS AND METHODS: This retrospective study was approved by our Institutional Review Board, and informed consent was waived. Microcatheter looping method was employed in 59 patients with paraclinoid aneurysms between January 2012 and December 2013. In the described technique, construction of a microcatheter loop, which is steam-shaped or pre-shaped, based on the direction of aneurysms, is mandatory. The looped tip of microcatheter was advanced into distal internal carotid artery and positioned atop the target aneurysm. By steering the loop (via inner microguidewire) into the dome of aneurysm and easing tension on the microcatheter, the aneurysm was selected. Clinical and morphologic outcomes were assessed with emphasis on technical aspects of the treatment. RESULTS: Through this looping technique, a total of 59 paraclinoid aneurysms were successfully treated. After aneurysm selection as described, single microcatheter technique (n = 25) was most commonly used to facilitate coiling, followed by balloon protection (n = 21), stent protection (n = 7), multiple microcatheters (n = 3), and stent/balloon combination (n = 3). Satisfactory aneurysmal occlusion was achieved through coil embolization in 44 lesions (74.6%). During follow-up of 53 patients (mean interval, 10.9 +/- 5.9 months), only one instance (1.9%) of major recanalization was observed. There were no complications related to microcatheter looping. CONCLUSION: This microcatheter looping method facilitates safe and effective positioning of microcatheter into domes of paraclinoid aneurysms during coil embolization when other traditional microcatheter selection methods otherwise fail.
Adult ; Carotid Artery, Internal/radiography/*surgery ; Catheterization/methods ; Cerebral Angiography/methods ; Embolization, Therapeutic/*methods ; Female ; Humans ; Intracranial Aneurysm/*therapy ; Male ; Middle Aged ; Retrospective Studies ; Stents

Mutations in the calreticulin gene, CALR, have recently been discovered in subsets of patients with essential thrombocythemia (ET) or primary myelofibrosis (PMF). We investigated Korean patients with ET and PMF to determine the prevalence, and clinical and laboratory correlations of CALR/JAK2/MPL mutations. Among 84 ET patients, CALR mutations were detected in 23 (27.4%) and were associated with higher platelet counts (P=0.006) and lower leukocyte counts (P=0.035) than the JAK2 V617F mutation. Among 50 PMF patients, CALR mutations were detected in 11 (22.0%) and were also associated with higher platelet counts (P=0.035) and trended to a lower rate of cytogenetic abnormalities (P=0.059) than the JAK2 V617F mutation. By multivariate analysis, triple-negative status was associated with shorter overall survival (HR, 7.0; 95% CI, 1.6-31.1, P=0.01) and leukemia-free survival (HR, 6.3; 95% CI, 1.8-22.0, P=0.004) in patients with PMF. The type 1 mutation was the most common (61.1%) type among all patients with CALR mutations, and tended toward statistical predominance in PMF patients. All 3 mutations were mutually exclusive and were never detected in patients with other myeloid neoplasms showing thrombocytosis. CALR mutations characterize a distinct group of Korean ET and PMF patients. Triple-negative PMF patients in particular have an unfavorable prognosis, which supports the idea that triple-negative PMF is a molecularly high-risk disease.
Adult ; Aged ; Aged, 80 and over ; Calreticulin/*genetics ; Disease-Free Survival ; Female ; Gene Frequency ; Genetic Association Studies ; Humans ; Janus Kinase 2/*genetics ; Male ; Middle Aged ; Mutation/genetics ; Primary Myelofibrosis/*genetics/mortality ; Receptors, Thrombopoietin/*genetics ; Republic of Korea ; Thrombocythemia, Essential/*genetics/mortality ; Young Adult

Although endobronchial hamartoma is a rare benign tumor, most patients with endobronchial hamartoma have respiratory symptoms such as obstructive pneumonia, hemoptysis, cough, or dyspnea due to bronchial obstruction. It can cause irreversible post-obstructive pulmonary destruction, thus early diagnosis and treatment is very important. Recently, there have been cases of neodymium-doped yttrium aluminum garnet (Nd:YAG) laser and electrocautery procedures for bronchoscopic treatment of malignant or benign central airway obstruction with comparable therapeutic efficacy and few complications. Bronchoscopic cryotherapy is a newly developed technique for management of central airway obstruction. Moreover, it provides diagnostic methods with improving diagnostic yield and safety. We report two cases of endobronchial hamartoma, each diagnosed and definitively treated with bronchoscopic techniques. Endobronchial biopsy and removal was successfully performed by cryotherapy via flexible bronchoscopy without notable complications. Follow-up bronchoscopic examinations excluded residual or recurrent disease.
Airway Obstruction ; Aluminum ; Biopsy ; Bronchial Neoplasms ; Bronchoscopy* ; Cough ; Cryotherapy* ; Diagnosis* ; Dyspnea ; Early Diagnosis ; Electrocoagulation ; Follow-Up Studies ; Hamartoma* ; Hemoptysis ; Humans ; Pneumonia ; Yttrium

BACKGROUND: The presence of significant dysplasia in bone marrow (BM) aspirates helps to distinguish between hypocellular myelodysplastic syndrome (hMDS) and aplastic anemia (AA). Occasionally, diluted BM aspirates make it difficult to recognize dysplastic changes and can also negatively affect the detection of cytogenetic abnormalities in hMDS. We evaluated the usefulness of CD34 and p53 immunoreactivity for discriminating between hMDS and AA and for estimating survival outcomes in hMDS patients. METHODS: BM clot section (BMC) or BM biopsy (BMB) specimens were obtained from 64 hMDS/AA patients (33 with hMDS and 31 with AA) and seven controls. Immunohistochemical (IHC) staining for CD34 and p53 was performed by using the EnVision detection system (Dako, Denmark). We compared the results of IHC staining, BM findings, and chromosomal analyses, and determined overall survival outcomes. RESULTS: The number of CD34- and p53-positive BM cells was higher among the patients with hMDS than among the patients with AA (P<0.001 and P=0.001, respectively). hMDS patients with increased CD34-positive cells had significantly poorer survival outcomes compared with those with normal number of CD34-positive cells (P=0.013). CONCLUSIONS: CD34 and p53 IHC stains of BMC or BMB provide useful information for differentiating between hMDS and AA. CD34 IHC staining of BMC or BMB also provides useful information for estimating survival outcomes in hMDS patients.
Adolescent ; Adult ; Anemia, Aplastic/*diagnosis ; Antigens, CD34/*metabolism ; Bone Marrow/metabolism/*pathology ; Child ; Chromosome Aberrations ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Myelodysplastic Syndromes/*diagnosis/mortality ; ROC Curve ; Tumor Suppressor Protein p53/*metabolism

Infantile hemangioma is a self-resolving benign tumor that commonly requires no intervention. Despite its distinctive, self-limiting course, infantile hemangiomas may result in ulcerations that cause pain, potential scarring, bleeding, and infection. Active treatment is usually required to manage these lesions. The discovery of propranolol in the treatment of infantile hemangioma was described in 2008 by Leaute-Labreze, et al. Since then, its effectiveness and safety have been confirmed by several cases, and replacing systemic corticosteroids as a first-line therapy. Herein, we report the successful treatment of children with ulcerated perianal infantile hemangioma with propranolol.
Adrenal Cortex Hormones ; Child ; Cicatrix ; Hemangioma ; Hemorrhage ; Humans ; Propranolol ; Ulcer

Washboard nail is a dystrophy that presents as a series of ridges that extends across the nail. It is often accompanied by a central depression that extends from the cuticle to the free edge. Washboard nail is often a result of frequently repeated trauma, but sometimes predisposing factors cannot be found. When habitual tic is the cause of the lesions, restraining the habit is the most important treatment. Here, we report a case of a 6-year-old male with washboard nails on both thumbnails and was treated with topical 0.1% tacrolimus ointment. Significant improvement was observed after 4 months of application. This case suggests that topical tacrolimus can be considered as a primary therapy in the treatment of washboard nails in the absence of trauma.
Depression ; Humans ; Male ; Nails ; Tacrolimus ; Tics