Purpose: Acute promyelocytic leukemia (APL) is a rare disease in children and there are some different characteristics between children and adult. We aimed to evaluate incidence, clinical characteristics and treatment outcomes of pediatric APL in Korea. Materials and Methods: Seventy-nine pediatric APL patients diagnosed from January 2009 to December 2016 in 16 tertiary medical centers in Korea were reviewed retrospectively. Results: Of 801 acute myeloid leukemia children, 79 (9.9%) were diagnosed with APL. The median age at diagnosis was 10.6 years (range, 1.3 to 18.0). Male and female ratio was 1:0.93. Thirty patients (38.0%) had white blood cell (WBC) count greater than 10×109/L at diagnosis. All patients received induction therapy consisting of all-trans retinoic acid and chemotherapy. Five patients (6.6%) died during induction chemotherapy and 66 patients (86.8%) achieved complete remission (CR) after induction chemotherapy. The causes of death were three intracranial hemorrhage, one cerebral infarction, and one sepsis. Five patients (7.1%) suffered a relapse during or after maintenance chemotherapy. The estimated 4-year event-free survival and overall survival (OS) rates were 82.1%±4.4%, 89.7%±5.1%, respectively. The 4-year OS was significantly higher in patients with initial WBC < 10×109/L than in those with initial WBC ≥ 10×109/L (p=0.020). Conclusion This study showed that the CR rates and survival outcomes in Korean pediatric APL patients were relatively good. The initial WBC count was the most important prognostic factor and most causes of death were related to serious bleeding in the early stage of treatment.

Background: Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA. Methods: We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey. Results: A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia. Conclusion In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.

Background: Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. Methods: We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. Results: A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. Conclusion This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.

Rapunzel syndrome is caused by gastric trichobezoar with extended tail and small bowel obstruction. Patients with gastric trichobezoar can be asymptomatic until the bezoar increases in size. We report a case of a girl who visited the emergency department with abdominal pain. She was finally diagnosed with Rapunzel syndrome that causes multiple small bowel intussusceptions associated with trichophagia. Surgery was needed to reduce the multiple intussusceptions, and to remove the large trichobezoar. This case highlights to consider the possibility of Rapunzel syndrome when diagnosing the main cause of intussusceptions.
Abdominal Pain ; Bezoars ; Emergency Service, Hospital ; Female ; Humans ; Intestinal Obstruction ; Intussusception ; Pica ; Tail ; Trichotillomania

BACKGROUND: von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. However, the number of patients who register to the Korea Hemophilia Foundation (KHF) is much lower than the expected prevalence rate and only few hospitals perform tests for diagnosis autonomously. Thus, we surveyed practical realities of VWD in Yeungnam region. METHODS: Patients with VWD (N=267) who were diagnosed at eleven university hospitals from March 1995 to March 2018 were enrolled in this study. We evaluated the medical records from each hospital retrospectively. RESULTS: Two hundred and twenty-eight children and 39 adults met the diagnostic criteria for VWD. Seventy-eight (57.4%) patients had the blood type O. Fifty-eight patients were definite type 1 (21.7%), 151 were possible type 1 (56.6%), and the others were type 2. Abnormal laboratory findings were the most common factor for the diagnosis in children. VWF mutations were detected in 17 patients. Patients with a family history showed age of diagnosis of 9 y, which is higher than in those with no family history (6 yr), and also showed a higher rate of significant bleeding (32.1% vs. 14.2%). VWF:RCo and VWF:Ag tests were performed in-hospital at only 1 of 11 hospitals. Twelve of 267 patients were enrolled at the KHF (4.5%). CONCLUSION: A high rate of out-sourcing studies may result in inaccurate diagnosis. The registration rate to the KHF is still lower than the prevalence rate. A comprehensive nationwide registration system is necessary in order to identify the actual prevalence rate and promote the diagnosis of VWD in Korea.
Adult ; Child ; Diagnosis ; Hemophilia A ; Hemorrhage ; Hospitals, University ; Humans ; Korea ; Medical Records ; Prevalence ; Retrospective Studies ; von Willebrand Diseases

OBJECTIVE: The aim of this study was to evaluate the clinical characteristics of heat stroke in a bath facility and investigate predictive factors of multiple major complications in heat stroke patients. METHODS: This was a retrospective study on heat stroke patients who visited an urban emergency center from January 2010 to March 2018. We compared clinical characteristics, complication, and outcomes of heat stroke patients in bath and non-bath facilities. Multivariate logistic regression analysis were performed to identify independent predictors of multiple major complications in heat stroke patients. RESULTS: A total of 67 heat stroke patients with heat stroke were enrolled, of which 42 (62.6%) were in a bath facility and 25 (37.3%) were in a non-bath facility. Patients with heat stroke in the bath facility were characterized by old age, past medical history of hypertension and diabetes mellitus, and high incidence of hypotension compared with those in the non-bath facility but also low incidence of acute renal failure, seizure, and multiple major complications. In the multivariate analysis, predictive factors of multiple major complications in heat stroke patients were non-bath facility (odds ratio [OR], 5.4; 95% confidence interval [CI], 1.2–29.9), Glasgow Coma Scale (GCS)≤8 (OR, 8.2; 95% CI, 1.3–49.4), and mean arterial pressure (MAP), body temperature above 40.5℃ (OR, 8.1; 95% CI, 1.1–58.8) < 60 mmHg (OR, 14.8; 95% CI, 1.8–122.9). CONCLUSION: Heat stroke in the bath facility resulted in less major complications, and high body temperature, GCS ≤8, and MAP < 60 mmHg were independent predictive factors of multiple major complications in heat stroke patients.
Acute Kidney Injury ; Arterial Pressure ; Baths* ; Body Temperature ; Diabetes Mellitus ; Emergencies ; Glasgow Coma Scale ; Heat Stroke* ; Hot Temperature* ; Humans ; Hypertension ; Hypotension ; Incidence ; Logistic Models ; Multivariate Analysis ; Retrospective Studies ; Seizures

The author's affiliation should be corrected. We apologize for any inconvenience that may have caused.

The author's affiliation should be corrected. We apologize for any inconvenience that may have caused.

Inflammatory myositis is a heterogeneous group of rare diseases characterized by inflammation of the skeletal muscle. The association between cancer and inflammatory myositis is well established, with most cancer-associated myopathies diagnosed within 2 years of initial diagnosis. However, despite this strong association, inflammatory myositis as a paraneoplastic syndrome of urethral cancer has not been reported in Korea. Furthermore, compartment syndrome in the context of inflammatory myositis is extremely rare. A 69 year-old woman presented with paresthesia and painful swelling of the right lower extremity 2 months after diagnosis with urethral cancer, which was treated by chemoradiotherapy. Painful numbness was particularly severe when extending the knee, leading to a preliminary diagnosis of myositis with compartment syndrome, which was confirmed by magnetic resonance imaging. After fascia resection, the patient was treated with high dose steroid and immunoglobulin therapy. Here, we report a case of compartment syndrome followed by inflammatory myositis in a patient with urethral cancer.
Chemoradiotherapy ; Compartment Syndromes* ; Diagnosis ; Fascia ; Female ; Humans ; Hypesthesia ; Immunization, Passive ; Inflammation ; Knee ; Korea ; Lower Extremity ; Magnetic Resonance Imaging ; Muscle, Skeletal ; Muscular Diseases ; Myositis* ; Paraneoplastic Syndromes ; Paresthesia ; Rare Diseases ; Urethral Neoplasms*