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1.  Langerhans Cell Sarcoma in Two Young Children: Imaging Findings on Initial Presentation and Recurrence 
Korean Journal of Radiology  2013;14(3):520-524.
Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells with malignant cytological features and multi-organ involvement that typically has a poor prognosis. We experienced 2 cases of LCS in children less than 2 years of age and report them based primarily on CT and MR findings. Both children had findings of hepatosplenomegaly with low-attenuation nodular lesions, had multiple lymphadenopathy, and had shown recurrent lesions invading the skull during follow-up after chemotherapy.
doi:10.3348/kjr.2013.14.3.520
PMCID: PMC3655310  PMID: 23690723
Langerhans cell sarcoma; Langerhans cell histiocytosis; CT; MR
2.  Acute Appendicitis in Children with Acute Leukemia: Experiences of a Single Institution in Korea 
Yonsei Medical Journal  2012;53(4):781-787.
Purpose
Acute appendicitis has been reported to be relatively rare in pediatric leukemia patients but there is no official data for this in Korea. And there is no consensus for its treatment in this population.
Materials and Methods
We conducted a retrospective study of 7 patients diagnosed with appendicitis among 1209 pediatric patients who were diagnosed with leukemia from 1996 to 2008 at a single institution in Korea.
Results
The median age at the time of the diagnosis of appendicitis was 12 years (range: 3-15 years), and 3 of the patients were male. The median absolute neutrophil count (ANC) at the time of diagnosis was 0.99×109/L (range: 0-3×109/L). The mean time from the onset of symptoms to the diagnosis was 4 days. All 7 leukemia patients with appendicitis underwent surgery and they demonstrated a survival of 100% without significant complications.
Conclusion
The incidence of appendicitis in pediatric leukemia patients was 0.57% in our study. Early diagnosis with abdominal ultrasound or computed tomography and early surgical resection in leukemic patient with acute appendicitis may be a safer and more effective treatment option. Even when perforation has already occurred and when the patient has an ANC of 0×109/L, surgical treatment may improve overall survival without incurring significant complications.
doi:10.3349/ymj.2012.53.4.781
PMCID: PMC3381485  PMID: 22665346
Acute appendicitis; leukemia; neutropenia; appendectomy
3.  Clinical and hematologic manifestations in patients with Diamond Blackfan anemia in Korea 
The Korean Journal of Hematology  2012;47(2):131-135.
Background
Diamond Blackfan anemia (DBA), characterized by impaired red cell production, is a rare condition that is usually symptomatic in early infancy. The purpose of this study was to assess nationwide experiences of DBA encountered over a period of 20 years.
Methods
The medical records of 56 patients diagnosed with DBA were retrospectively reviewed from November 1984 to July 2010. Fifteen institutions, including 13 university hospitals, participated in this study.
Results
The male-to-female ratio of patients with DBA was 1.67:1. The median age of diagnosis was 4 months, and 74.1% were diagnosed before 1 year of age. From 2000 to 2009, annual incidence was 6.6 cases per million. Excluding growth retardation, 38.2% showed congenital defects: thumb deformities, ptosis, coarctation of aorta, ventricular septal defect, strabismus, etc. The mean hemoglobin concentration was 5.1±1.9 g/dL, mean corpuscular volume was 93.4±11.6 fL, and mean number of reticulocytes was 19,700/mm3. The mean cellularity of bone marrow was 75%, with myeloid:erythroid ratio of 20.4:1. After remission, 48.9% of patients did not need further steroids. Five patients with DBA who received hematopoietic transplantation have survived. Cancer developed in 2 cases (3.6%).
Conclusion
The incidence of DBA is similar to data already published, but our study had a male predilection. Although all patients responded to initial treatment with steroids, about half needed further steroids after remission. It is necessary to collect further data, including information regarding management pathways, from nationwide DBA registries, along with data on molecular analyses.
doi:10.5045/kjh.2012.47.2.131
PMCID: PMC3389062  PMID: 22783360
Diamond Blackfan anemia; Anemia; Congenital defects
4.  Assessment of Serologic Immunity to Diphtheria-Tetanus-Pertussis After Treatment of Korean Pediatric Hematology and Oncology Patients 
The aim of this study was to investigate the diphtheria-tetanus-pertussis antibody titers after antineoplastic treatment and to suggest an appropriate vaccination approach for pediatric hemato-oncologic patients. A total of 146 children with either malignancy in remission after cessation of therapy or bone marrow failure were recruited. All children had received routine immunization including diphtheria-tetanus-acellular pertussis vaccination before diagnosis of cancer. The serologic immunity to diphtheria, tetanus and pertussis was classified as: completely protective, partially protective, or non-protective. Non-protective serum antibody titer for diphtheria, tetanus and pertussis was detected in 6.2%, 11.6%, and 62.3% of patients, respectively, and partial protective serum antibody titer for diphtheria, tetanus and pertussis was seen in 37%, 28.1%, and 8.9% of patients. There was no significant correlation between the severity of immune defect and age, gender or underlying disease. Revaccination after antineoplastic therapy showed significantly higher levels of antibody for each vaccine antigen. Our data indicates that a large proportion of children lacked protective serum concentrations of antibodies against diphtheria, tetanus, and pertussis. This suggests that reimmunization of these patients is necessary after completion of antineoplastic treatment. Also, prospective studies should be undertaken with the aim of devising a common strategy of revaccination.
doi:10.3346/jkms.2012.27.1.78
PMCID: PMC3247779  PMID: 22219618
Serologic Immunity; Immunocompromised Children; Diphtheria; Tetanus; Pertussis; Vaccination
5.  The role of B cells in acute graft-versus-host disease 
The Korean Journal of Hematology  2011;46(4):287-288.
doi:10.5045/kjh.2011.46.4.287
PMCID: PMC3259523  PMID: 22259637
6.  Development of Bullous Acrodermatitis Enteropathica during the Course of Chemotherapy for Acute Lymphocytic Leukemia 
Annals of Dermatology  2011;23(Suppl 3):S326-S328.
Acrodermatitis enteropathica (AE) is an uncommon autosomal recessive genetic disorder of zinc malabsorption. The acquired form may be associated with inadequate intake, impaired absorption, and increased excretion of zinc. Those afflicted present with diarrhea, stomatitis, psychiatric symptoms, non-scarring alopecia, and nail dystrophy accompanied by erythematous which appears as scaly patches with erosion vesicles and pustules mostly affecting the extremities, perineal, and periorificial areas. Due to the variable findings of most case reports, the clinical and histopathological features of AE are often regarded as non-specific. We report an unusual case of bullous AE secondary to total parenteral nutrition for the treatment of acute pancreatitis occurring in a six-year-old male with acute lymphocytic leukemia who underwent chemotherapy. He presented with periorificial, reddish, eroded bullae with multiple vesicles and blisters on his fingers, toes, and buttock, showing necrotic keratinocytes with multiple intraepidermal vesicles and perivascular infiltration with predominant lymphocytes and few neutrophils within the dermis. To the best of our knowledge, this is the first case report of bullous AE in the Korean dermatologic literature.
doi:10.5021/ad.2011.23.S3.S326
PMCID: PMC3276788  PMID: 22346269
Bullous acrodermatitis enteropathica; Chemotherapy; Total parenteral nutrition
7.  Outcome of childhood acute promyelocytic leukemia treated using a modified AIDA protocol 
The Korean Journal of Hematology  2010;45(4):236-241.
Background
Combination treatment with all-trans-retinoic acid (ATRA) and anthracycline-based chemotherapy has led to major advances in the treatment of acute promyelocytic leukemia (APL).
Methods
In this study, we reviewed the outcome of pediatric APL patients treated using a modified AIDA protocol at our institution.
Results
Between May 1999 and December 2007, 23 patients were diagnosed with APL at the Department of Pediatrics, Saint Mary's Hospital, The Catholic University of Korea. Eleven patients were male (48%) (median age at diagnosis, 11 (range, 2-14) years). The treatment protocol consisted of remission induction (achieved by coadministration of ATRA and idarubicin), 3 courses of consolidation treatment, and 2 years of maintenance treatment during which ATRA was also administered. Three patients died early during remission induction due to CNS hemorrhage. The remaining 20 patients achieved complete remission (CR), with an overall CR rate of 87%. Two patients relapsed and died, and another patient died of pneumonia unrelated to APL. Four patients (17%) were diagnosed with ATRA syndrome, and all patients showed resolution of symptoms. The event-free survival (EFS) and overall survival (OS) of the cohort were 78.3±8.6% and 76.3±9.5%, respectively. Initial WBC count at diagnosis was the only significant prognostic factor for the rate of CR (P=0.039) and OS (P=0.039).
Conclusion
A modified AIDA protocol for the treatment of childhood APL leads to improved EFS and OS, with limited ATRA syndrome-associated toxicity. Active monitoring and treatment of patients with high initial WBC counts may help in reducing mortality.
doi:10.5045/kjh.2010.45.4.236
PMCID: PMC3023048  PMID: 21253424
Acute promyelocytic leukemia; Children; All-trans-retinoic acid; Anthracycline
8.  Lymphomatoid Papulosis Followed by Anaplastic Large Cell Lymphoma in a Pediatric Patient 
Annals of Dermatology  2010;22(4):447-451.
Lymphomatoid papulosis (LyP) is a benign, self-healing, papular eruption that can wax and wane over time. Transformation to T-cell lymphoma has been well documented in 10% to 20% of adults with LyP. However, this transformation rarely occurs in patients younger than 20 years of age. Here, we present the first known pediatric patient in Korea, a 12-year-old boy who developed a subcutaneous nodule on the scrotum 13 months after papulonecrotic lesions of LyP were identified on both lower extremities and face. Histological and immunohistochemical examination of the subcutaneous nodule revealed anaplastic large cell lymphoma (ALCL). A T-cell receptor gene rearrangement analysis demonstrated an identical rearranged pattern in the two specimens, indicating that a common T-cell clone had proliferated over time in both the LyP and ALCL lesions.
doi:10.5021/ad.2010.22.4.447
PMCID: PMC2991725  PMID: 21165218
Anaplastic large cell lymphoma; Lymphomatoid papulosis
9.  Efficacy and safety of deferiprone (Ferriprox), an oral iron-chelating agent, in pediatric patients 
Background
Iron overload is a predictable and life-threatening complication in patients dependent on the regular transfusion of RBCs. The aims of this study were to investigate the efficacy and safety of deferiprone in a variety of pediatric hematologic and/or oncologic patients with a high iron overload.
Methods
Seventeen patients (age: 1.1-20.4 years; median: 10.6 years) from 7 hospitals who were treated with deferiprone from 2006 to 2009 were enrolled in this study. Medical records of enrolled patients were reviewed retrospectively.
Results
Serum ferritin levels were 4,677.8±1,130.9 µg/L at baseline compared to 3,363.9±1,149.7 µg/L at the end of deferiprone treatment (P=0.033). Only 1 patient developed neutropenia as a complication.
Conclusion
Deferiprone treatment is relatively safe for pediatric patients suffering from various hematologic and oncologic diseases that require RBC transfusions as part of treatment. However, the potential development of critical complications such as agranulocytosis and/or neutropenia remains a concern.
doi:10.5045/kjh.2010.45.1.58
PMCID: PMC2983007  PMID: 21120164
Deferiprone; Iron overload; Transfusion; Neutropenia
10.  Neostigmine for the treatment of acute colonic pseudo-obstruction (ACPO) in pediatric hematologic malignancies 
Background
Acute colonic pseudo-obstruction (ACPO) refers to dilatation of the colon and decreased bowel motility without evidence of mechanical obstruction. Neostigmine, an acetylcholinesterase inhibitor, has been used in patients in whom supportive therapy failed to resolve ACPO. Here, we report the results of administering neostigmine to treat ACPO in children with hematologic malignancies.
Methods
Between September 2005 and December 2009, 10 patients (8 male and 2 female) were diagnosed with ACPO at the Department of Pediatrics, Catholic University of Korea. Diagnosis of ACPO was based on typical clinical features as well as colonic dilatation found on abdominal CT imaging. Neostigmine was administered subcutaneously at a dosage of 0.01 mg/kg/dose (maximum 0.5 mg) twice daily for a maximum of 5 total doses. ACPO was determined to be responsive to neostigmine if the patient showed both stool passage and improvement of clinical symptoms.
Results
The study group included 8 acute lymphoblastic leukemia patients, 1 patient with malignant lymphoma, and 1 patient with juvenile myelomonocytic leukemia. The median age at ACPO diagnosis was 8.5 years (range, 3-14). Overall, 8 patients (80%) showed therapeutic response to neostigmine at a median of 29 hours after the initial administration (range, 1-70). Two patients (20%) showed side effects of grade 2 or above, but none complained of cardiovascular symptoms that required treatment.
Conclusion
In this study, ACPO was diagnosed most often in late-childhood ALL patients. Subcutaneous neostigmine can be used to effectively treat ACPO diagnosed in children with hematologic malignancies without major cardiovascular complications.
doi:10.5045/kjh.2010.45.1.62
PMCID: PMC2983008  PMID: 21120165
Acute colonic pseudo-obstruction; Neostigmine; Children; Hematologic malignancies
11.  Idarubicin Plus Behenoyl Cytarabine and 6-thioguanine Compares Favorably with Idarubicin Plus Cytarabine-based Regimen for Children with Previously Untreated Acute Myeloid Leukemia: 10-Year Retrospective, Multicenter Study in Korea 
We investigated the outcome of idarubicin plus N4-behenoyl-1-β-D-arabinofuranosyl cytosine (BHAC)-based chemotherapy (BHAC group, n=149) compared to idarubicin plus cytarabine-based chemotherapy (cytarabine group, n=191) for childhood acute myeloid leukemia (AML). Between January 1996 and December 2005, 340 children with AML from 5 university hospitals in Korea received the BHAC-based or cytarabine-based chemotherapy, with or without hematopoietic stem cell transplantation. After induction therapy, 264 (77.6%) of 340 children achieved a complete remission (CR) and 43 (12%) achieved a partial remission (PR). The CR rate in the BHAC group was higher than in the cytarabine group (85.2% vs. 71.7%, P=0.004). However, the overall response rate (CR+PR) was not different between the two groups (93.3% vs. 87.9%, P=0.139). The 5-yr estimates of overall survival (OS) of children in the two groups were similar (54.9% for the BHAC group vs. 52.4% for the cytarabine group, P=0.281). Although the results were analyzed according to the treatment type and cytogenetic risk, the OS showed no significant difference between the BHAC group and the cytarabine group. In the present study, the clinical outcomes of the BHAC-based chemotherapy, consisting of BHAC, idarubicin, and 6-TG, are comparable to that of the cytarabine-based chemotherapy for childhood AML.
doi:10.3346/jkms.2010.25.1.9
PMCID: PMC2800026  PMID: 20052341
Leukemia, Myeloid, Acute; Enocitabine; Childhood
12.  Kikuchi's Disease in Children: Clinical Manifestations and Imaging Features 
Journal of Korean Medical Science  2009;24(6):1105-1109.
Previously published studies on Kikuchi disease (KD) have frequently addressed the computed tomography (CT) findings in the adult population, however, only a few studies have been reported for the pediatric age group. The purpose of this study is to analyze the clinical characteristics and imaging features of KD in children. Fifteen children (2-14 yr) who had a neck CT and pathology diagnosis of KD were included in this study. Clinical features, including the duration of lymphadenopathy and fever, prognosis, and laboratory values, were evaluated. We analyzed the sites, size, and lymph node pattern as seen on their CT scans. The median duration of fever was 10 days. Fourteen patients experienced improvement in their condition, although four of these patients experienced recurrent episodes of KD. All patients had affected cervical nodes at level V. Perinodal infiltrates were observed in the affected cervical nodes in 14 cases (93%), and non-enhancing necrosis was also noted within the affected cervical nodes in 10 cases (63%). In conclusion, the combination of imaging findings in conjunction with clinical findings of KD may help to determine whether or not to perform pathology analysis and follow-up studies.
doi:10.3346/jkms.2009.24.6.1105
PMCID: PMC2775859  PMID: 19949667
Lymphatic Diseases; Histiocytic Necrotizing Lymphadenitis; Child
13.  Endocrine Complications after Hematopoietic Stem Cell Transplantation during Childhood and Adolescence 
Journal of Korean Medical Science  2009;24(6):1071-1077.
Long-term survivors of hematopoietic stem cell transplantation (HSCT) during childhood and adolescence are at risk of developing endocrine complications. The purpose of this study was to evaluate the long-term endocrine complications and their associated risk factors among such patients. We reviewed the data from 111 patients (59 males and 52 females) who underwent HSCT at the mean age of 8.3±4.1 yr. Thirty patients (27.0%) had growth impairment, and seven (21.2%) out of 33 patients who attained final height reached final height below 2 standard deviation (SD). The final height SD score of the patients conditioned with total body irradiation (TBI) was significantly lower than that of the patients conditioned without TBI (-1.18±1.14 vs. -0.19±0.78, P=0.011). Thirteen patients (11.7%) developed hypothyroidism (11 subclinical, 2 central) 3.8±1.8 (range 1.6-6.2) yr after HSCT. Nineteen (65.5%) out of 29 females had evidence of gonadal dysfunction, and 18 (64.3%) out of 28 males had evidence of gonadal dysfunction. The risk for gonadal dysfunction was significantly higher in females conditioned with busulfan/cyclophosphamide (P=0.003). These results suggest that the majority of patients treated with HSCT during childhood and adolescence have one or more endocrine complications. Therefore, multiple endocrine functions should be monitored periodically after HSCT until they reach adult age.
doi:10.3346/jkms.2009.24.6.1071
PMCID: PMC2775854  PMID: 19949662
Endocrine Complications; Transplantation; Childhood
14.  Gastrointestinal Complications Following Hematopoietic Stem Cell Transplantation in Children 
Korean Journal of Radiology  2008;9(5):449-457.
Gastrointestinal system involvement is one of the principal complications seen in the recipients of hematopoietic stem cell transplantation (HSCT), and it is also a major cause of morbidity and death in these patients. The major gastrointestinal complications include typhlitis (neutropenic enterocolitis), pseudomembranous enterocolitis, viral enteritis, graft-versus-host disease, benign pneumatosis intestinalis, intestinal thrombotic microangiopathy, and post-transplantation lymphoproliferative disease. As these patients present with nonspecific abdominal symptoms, evaluation with using such imaging modalities as ultrasonography and CT is essential in order to assess the extent of gastrointestinal involvement and to diagnose these complications. We present here a pictorial review of the imaging features and other factors involved in the diagnosis of these gastrointestinal complications in pediatric HSCT recipients.
doi:10.3348/kjr.2008.9.5.449
PMCID: PMC2627203  PMID: 18838855
Hematopoietic stem cell transplantation; Pediatric; Complications; Gastrointestinal tract
15.  Co-transplantation of Human Mesenchymal Stem Cells Promotes Human CD34+ Cells Engraftment in a Dose-dependent Fashion in NOD/SCID Mice 
Journal of Korean Medical Science  2007;22(3):412-419.
Mesenchymal stem cells (MSCs) have recently been identified and characterized in humans. Moreover, MSC secrete cytokines that can support hematopoietic progenitor growth. In the present study, we evaluated whether the efficacy of hematopoietic stem cell transplantation is improved by their co-transplantation with MSC, and whether this is positively correlated with the dose of infused MSCs. Accordingly, irradiated NOD/SCID mice were transplanted with 1×105 human CD34+ cells in the presence or absence of culture expanded MSCs (1×106 or 5×106). We evaluated human hematopoietic cell engraftment by flow cytometry and assessed MSC tissue distributions by fluorescence in situ hybridization. We found that CD45+ and CD34+ cell levels were significantly elevated in a dose-dependent manner in cotransplanted mice 4 weeks after transplantation. The engraftments of CD33+ and CD19+ cells also increased dose-dependently. However, the engraftment of CD3+ cells did not increase after co-transplantation with MSCs. Human Y chromosome+ cells were observed in multiple tissues and were more frequently observed in mice co-transplanted with 5×106 rather than 1×106 MSCs. These results suggest that MSCs are capable of enhancing hematopoietic cell engraftment and distribution in multiple organs in a dose-dependent fashion.
doi:10.3346/jkms.2007.22.3.412
PMCID: PMC2693630  PMID: 17596646
Mesenchymal Stem Cells; Hematopoietic Stem Cells; Transplantation

Results 1-15 (15)