1.Bronchiolitis Obliterans Syndrome after Allogenic Hematopoietic Stem Cell Transplantation in Pediatric Patients
Ju Yeon LIM ; Seung Min HAHN ; Hyo Sun KIM ; Jung Woo HAN ; Chuhl Joo LYU
Clinical Pediatric Hematology-Oncology 2015;22(2):127-135
BACKGROUND: Bronchiolitis obliterans syndrome (BOS) is a life-threatening lung complication after allogenic hematopoietic stem cell transplantation (HSCT). As long-term survival following allogenic HSCT has improved, the number of BOS patients has been steadily increased. However, the survival and treatment of BOS have not improved significantly for decades. Identification of risk factors of BOS would improve the clinical outcome of allogenic HSCT recipients.METHODS: We retrospectively investigated medical records of 147 allogenic HSCT recipients between 2005 and 2014 in Yonsei Cancer Center. Risk factors for BOS were analyzed with Chi-square test, logistic regression analysis, and the Student's t-test.RESULTS: BOS occurred to 23 patients (15.6%). Pulmonary function test (PFT) results before transplantation were similar in all patients, but patients with BOS had a significant decrease in forced expiratory volume in one second (FEV1) after transplantation compared with controls (68.4+/-26.4% vs. 91.6+/-21.0%, P<0.05). Acute graft-versus-host disease (GVHD) (OR 5.98, P=0.009) and peripheral blood as sources of stem cell (OR 4.00, P=0.031) increased risk for BOS, respectively. On the other hand, previously reported risk factors, such as age of donors and recipients, pulmonary infection within 100 days after allogenic HSCT and deference of immunosuppressant were not associated with increased the incidence of BOS in our study.CONCLUSION: We report here the result of a single-center study on the incidence, clinical factors, and outcome of BOS after allogenic HSCT. BOS is an important cause of post-transplantation morbidity and mortality. Risk reduction can be achieved by better prevention and control of BOS.
Bronchiolitis Obliterans
;
Bronchiolitis
;
Forced Expiratory Volume
;
Graft vs Host Disease
;
Hand
;
Hematopoietic Stem Cell Transplantation
;
Hematopoietic Stem Cells
;
Humans
;
Incidence
;
Logistic Models
;
Lung
;
Medical Records
;
Mortality
;
Respiratory Function Tests
;
Retrospective Studies
;
Risk Factors
;
Risk Reduction Behavior
;
Stem Cells
;
Tissue Donors
2.A Case of Hodgkin Lymphoma Developed in an Ataxia-telangiectasia Patient
Ju Yeon LIM ; Mina JEON ; Hyo Sun KIM ; Jung Woo HAN ; Chuhl Joo LYU ; Seung Min HAHN
Clinical Pediatric Hematology-Oncology 2016;23(2):184-187
Ataxia-telangiectasia (AT) is characterized by cerebellar ataxia, progressive immunodeficiency, radiation sensitivity, telangiectasia, and predisposition to malignancy. AT patients have a 100-fold increased risk for the development of lymphoid malignancies. It is important to consider AT in a child with pre-existing ataxia, or lymphoid malignancy that was diagnosed at a younger age than expected. This consideration avoids the confusion between ataxia development and toxicity from chemotherapy. Hodgkin's lymphoma (HL) is usually treated with chemotherapy and/or radiotherapy. Unfortunately, when treated with conventional doses of radiotherapy, AT patients invariably experience devastating necrosis of their normal tissues. Therefore, a new treatment protocol for patients with HL in AT must be established. In this paper, we report the case of an 8-year-old female patient with HL in AT who was treated with chemotherapy. This patient was also treated with brentuximab (which targets CD30) for salvage therapy after the disease progressed.
Ataxia
;
Ataxia Telangiectasia
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Cerebellar Ataxia
;
Child
;
Clinical Protocols
;
Drug Therapy
;
Female
;
Hodgkin Disease
;
Humans
;
Necrosis
;
Radiation Tolerance
;
Radiotherapy
;
Salvage Therapy
;
Telangiectasis
3.Expression of Phosphatase and Tensin Homologue, phospho-Akt, and p53 in Acral Benign and Malignant Melanocytic Neoplasms (Benign Nevi, Dysplastic Nevi, and Acral Melanomas).
So Min LYU ; Ju Yeon WU ; Ji Yeon BYUN ; Hae Young CHOI ; Sang Hee PARK ; You Won CHOI
Annals of Dermatology 2016;28(5):548-554
BACKGROUND: The role of the phosphatidylinositol-3 kinase signaling pathway in the development of acral melanoma has recently gained evidence. Phosphatase and tensin homologue (PTEN), one of the key molecules in the pathway, acts as a tumor suppressor through either an Akt-dependent or Akt-independent pathway. Akt accelerates degradation of p53. OBJECTIVE: We assessed the expression of PTEN, phospho-Akt (p-Akt), and p53 by immunohistochemistry in benign acral nevi, acral dysplastic nevi, and acral melanomas in the radial growth phase and with a vertical growth component. METHODS: Ten specimens in each group were included. Paraffin-embedded specimens were immunostained with antibodies for PTEN, p-Akt, and p53. We scored both the staining intensity and the proportion of positive cells. The final score was calculated by multiplying the intensity score by the proportion score. RESULTS: All specimens of benign acral nevi except one showed some degree of PTEN-negative cells. The numbers of p-Akt and p53-positive cells were higher in acral dysplastic nevi and melanoma than in benign nevi. P-Akt scores were 1.7, 1.8, 2.6, and 4.4, and p53 scores were 2.0, 2.1, 3.8, and 4.1 in each group. PTEN and p-Akt scores in advanced acral melanoma were higher than in the other neoplasms. CONCLUSION: The expression of PTEN was decreased and the expression of p-Akt was increased in acral melanoma, especially in advanced cases. The PTEN-induced pathway appears to affect the late stage of melanomagenesis. Altered expression of p-Akt is thought to be due to secondary changes following the loss of PTEN.
Antibodies
;
Dysplastic Nevus Syndrome*
;
Immunohistochemistry
;
Melanoma
;
Nevus*
;
Phosphotransferases
4.Adverse Factors and the Role of Cisplatin and Vinca Alkaloids for Hearing Impairment in Childhood Cancer Patients and Survivors
Min Woo HUR ; Seung Min HAHN ; In Seok MOON ; Ju Yeon LIM ; Seul Mi LEE ; Chuhl Joo LYU ; Jung Woo HAN
Clinical Pediatric Hematology-Oncology 2017;24(2):121-129
BACKGROUND: Although combined chemotherapy has increased survival rates among children with cancer, such treatments can induce sensorineural hearing loss. Therefore, we aimed to identify risk factors for hearing impairments in patients with childhood cancer.METHODS: Audiograms were obtained from 115 patients with childhood cancer and survivors (age < 20 years). Pure tone audiometry (PTA) was performed at octave intervals within the range of 250-8000 Hz. We evaluated clinical risk factors associated with hearing impairments. Hearing loss was evaluated based on the maximal decibel (dB) loss in any frequency for each ear (RA(max) or LA(max)) and weighted mean dB loss for specific frequencies (RA(avg) or LA(avg)).RESULTS: Forty percent of patients (N=46) exhibited hearing loss >20 dB based on the weighted mean value in either ear. Severe hearing impairments were observed in 56% of patients with brain tumors. Although cisplatin or vinca alkaloids were significant risk factors for hearing impairment, the use of both cisplatin and vinca alkaloids exhibited the highest odds ratio for hearing impairment (P < 0.001, < 0.001 for R/LA(max); P=0.099, 0.039 for R/LA(avg)). Multivariate analysis revealed that the use of both cisplatin and vinca alkaloids was an independent risk factor for hearing impairment based on RA(max), LA(max), and LA(avg) (P < 0.001, < 0.001, 0.039, respectively).CONCLUSION: Our findings indicate that cisplatin and vinca alkaloids exert an additive effect on the risk of hearing impairment in survivors of childhood cancer. Further prospective studies are thus required to determine the most effective chemotherapeutic regimen for reducing ototoxicity.
Audiometry
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Brain Neoplasms
;
Child
;
Cisplatin
;
Drug Therapy
;
Ear
;
Hearing Loss
;
Hearing Loss, Sensorineural
;
Hearing
;
Humans
;
Multivariate Analysis
;
Odds Ratio
;
Prospective Studies
;
Risk Factors
;
Survival Rate
;
Survivors
;
Vinca Alkaloids
;
Vinca
5.Etiologic Distribution, Diagnostic Tests and Treatment in Prosp??ectively Registered Patients with DILD for Two Years in a Tertiary Medical Center.
Yong Soo KWON ; Man Pyo CHUNG ; Gyeong Man JEON ; Yeon Ju LYU ; Chang Min YU ; Jae Chul CHOI ; Eun Hae KANG ; Won Jung KOH ; Gee Young SUH ; Hojoong KIM ; O Jung KWON
Tuberculosis and Respiratory Diseases 2005;58(6):570-575
INTRODUCTION: Diffuse interstitial lung diseases (DILD) comprise of a large group of lung diseases with diverse etiologies. They are classified into four categories based on the etiology and pathological findings. In Korea, epidemiological data on DILD has never been reported in a prospective manner. METHOD: From May 2002 to April 2004, total 487 patients with DILD were prospectively registered at Samsung Medical Center. The prospective observational analysis of the etiologies, its classification based on 2002 ATS/ERS (American Thoracic Society/European Respiratory Society) guidelines, as well as diagnostic tests and the retrospective analysis of the treatment modalities were carried out. Any infectious and malignant causes were excluded. RESULTS: 1) The patients were classified into idiopathic interstitial pneumonia (IIP) in 269 patients (55.2%), known causes of DILD in 168 patients (34.5%), sarcoidosis in 27 patients (5.5%), other forms of DILD in 14 patients (2.9%), and undetermined DILD in 9 patients (1.9%). 2) The diagnostic test showed that most patients had undergone chest high resolution computed tomography (HRCT) and pulmonary function test (PFT) (97%, 89%). Transbronchial lung biopsy (TBLB) and surgical lung biopsy (SLB) were performed in limited patients (38%, 29%). 3) Among 269 patients with IIP, 220 (82%) had idiopathic pulmonary fibrosis (IPF) while 23 (9%) had nonspecific interstitial pneumonia. SLB was carried out in 36% of patients with IIP. 4) Symptomatic supportive care was given to 67% of IPF, but specific medical treatment including corticosteroids was administered to 89% of non-IPF patients. CONCLUSION: A nationwide registry of DILD patients is required to determine the annual incidence, etiology, and practice pattern of diagnosis and treatment in Korea.
Adrenal Cortex Hormones
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Biopsy
;
Classification
;
Diagnosis
;
Diagnostic Tests, Routine*
;
Humans
;
Idiopathic Interstitial Pneumonias
;
Idiopathic Pulmonary Fibrosis
;
Incidence
;
Korea
;
Lung
;
Lung Diseases
;
Lung Diseases, Interstitial
;
Respiratory Function Tests
;
Retrospective Studies
;
Sarcoidosis
;
Thorax
6.Hereditary Hemolytic Anemia in Korea: a Retrospective Study from 1997 to 2006.
Hee Soon CHO ; Jeong Ok HAH ; Im Ju KANG ; Hyung Jin KANG ; Jae Yong KWAK ; Hong Hoe KOO ; Hoon KOOK ; Byoung Kook KIM ; Soon Ki KIM ; Seung Taik KIM ; Young Dae KIM ; Ji Yoon KIM ; Chul Soo KIM ; Thad GHIM ; Heung Sik KIM ; Sang Gyu PARK ; Seon Yang PARK ; Jun Eun PARK ; Soo Mee BANG ; Jong Jin SEO ; Chang In SUH ; Sang Kyun SOHN ; Ho Jin SHIN ; Hee Young SHIN ; Hyo Sup AHN ; Doyeun OH ; Eun Sun YOO ; Chuhl Joo LYU ; Sung Soo YOON ; Kun Soo LEE ; Kwang Chul LEE ; Kee Hyun LEE ; Soon Yong LEE ; Young Ho LEE ; Jung Ae LEE ; Jong Seok LEE ; Young Tak LIM ; Jae Young LIM ; Ho Joon IM ; Dae Chul JEONG ; So Young CHONG ; Joo Seop CHUNG ; Hye Lim JUNG ; Goon Jae CHO ; Deog Yeon JO ; Jong Youl JIN ; Eun Jin CHOI ; Myung Soo HYUN ; Pyung Han HWANG
Korean Journal of Hematology 2007;42(3):197-205
BACKGROUND: The aim of this study was to investigate the prevalence, clinical and laboratory findings of hereditary hemolytic anemia (HHA) in Korea from 1997 to 2006 and to develop the appropriate diagnostic approach for HHA. METHODS: By the use of questionnaires, information on the clinical and laboratory findings ofHHA diagnosed from 1997 to 2006 in Korea was collected and analyzed retrospectively. A total of 431 cases were enrolled in this study from 46 departments of 35 hospitals. RESULTS: The overall frequency of HHA did not change through the 10-year period for pediatrics but did show an increasing tendency for internal medicine. The overall male to female sex ratio did not show sex predominance (1.17:1), but a significant male predominance with a ratio of 1.49:1 was seen for pediatrics while a significant female predominance with a ratio of 1:1.97 was seen forinternal medicine. Of the total cases, 74.2% (282/431) were diagnosed before the age of 15 years. The etiologies of HHA were classified as red cell membrane defects, hemoglobinopathies, red cell enzyme deficiencies and unknown causes. There were 382 cases (88.6%) of red cell membrane defects with 376 cases (87.2%) of hereditary spherocytosis and 6 cases (1.4%) of hereditary elliptocytosis, 20 cases (4.6%) of hemoglobinopathies with 18 cases (4.2%) of beta-thalassemia, a case (0.2%) of alpha-thalassemia and a case (0.2%) of Hemoglobin Madrid, 7 cases (1.6%) of red cell enzyme deficiencies with 5 cases (1.2%) of glucose-6- phosphate dehydrogenase (G-6-PD) deficiency, a case (0.2%) of pyruvate kinase (PK) deficiency and a case (0.2%) of enolase deficiency, and 22 cases (5.1%) of unknown causes. The most common chief complaint in pediatric patients was pallor and that in adult patients was jaundice. In the red cell membrane defect group of patients, the level of hemoglobin was significantly higher than in adult patients. The mean corpuscular volume, mean corpuscular hemoglobin, corrected reticulocyte count, total and indirect bilirubin level and lactate dehydrogenase levels in the hemoglobinopathy group of patients were significantly lower than the values in the red cell membrane defect group of patients. The mean concentration of G-6-PD was 0.8+/-0.7U/1012RBC in the G-6-PD deficient patients, PK was 1.7U/1010 RBC in the PK deficient patient, and the level of enolase was 0.04U/g of Hb in the enolase deficient patient. CONCLUSION: The most prevalent cause of HHA in Korea during 1997 to 2006 was hereditary spherocytosis, but HHA by other causes such as hemoglobinopathy and red cell enzyme deficiency gradually increased with the development of molecular diagnostic methods and increasing general interest. However, the etiologies of HHA need to be pursued further in 5.1% of the patients. An systematic standard diagnostic approach is needed in a nationwide prospective study for correct diagnoses and appropriate management of HHA.
Adult
;
alpha-Thalassemia
;
Anemia, Hemolytic, Congenital*
;
beta-Thalassemia
;
Bilirubin
;
Cell Membrane
;
Diagnosis
;
Elliptocytosis, Hereditary
;
Erythrocyte Indices
;
Female
;
Hemoglobinopathies
;
Humans
;
Internal Medicine
;
Jaundice
;
Korea*
;
L-Lactate Dehydrogenase
;
Male
;
Oxidoreductases
;
Pallor
;
Pathology, Molecular
;
Pediatrics
;
Phosphopyruvate Hydratase
;
Prevalence
;
Pyruvate Kinase
;
Reticulocyte Count
;
Retrospective Studies*
;
Sex Ratio
;
Surveys and Questionnaires
7.Erratum: Correction of Affiliations in the Article “Clinical Characteristics and Treatment Outcomes in Children, Adolescents, and Young-adults with Hodgkin's Lymphoma: a KPHOG Lymphoma Working-party, Multicenter, Retrospective Study”
Jae Min LEE ; Jung Yoon CHOI ; Kyung Taek HONG ; Hyoung Jin KANG ; Hee Young SHIN ; Hee Jo BAEK ; Hoon KOOK ; Seongkoo KIM ; Jae Wook LEE ; Nack-Gyun CHUNG ; Bin CHO ; Seok-Goo CHO ; Kyung Mi PARK ; Eu Jeen YANG ; Young Tak LIM ; Jin Kyung SUH ; Sung Han KANG ; Hyery KIM ; Kyung-Nam KOH ; Ho Joon IM ; Jong Jin SEO ; Hee Won CHO ; Hee Young JU ; Ji Won LEE ; Keon Hee YOO ; Ki Woong SUNG ; Hong Hoe KOO ; Kyung Duk PARK ; Jeong Ok HAH ; Min Kyoung KIM ; Jung Woo HAN ; Seung Min HAHN ; Chuhl Joo LYU ; Ye Jee SHIM ; Heung Sik KIM ; Young Rok DO ; Jae Won YOO ; Yeon Jung LIM ; In-Sang JEON ; Hee won CHUEH ; Sung Yong OH ; Hyoung Soo CHOI ; Jun Eun PARK ; Jun Ah LEE ; Hyeon Jin PARK ; Byung-Kiu PARK ; Soon Ki KIM ; Jae Young LIM ; Eun Sil PARK ; Sang Kyu PARK ; Eun Jin CHOI ; Young Bae CHOI ; Jong Hyung YOON ;
Journal of Korean Medical Science 2021;36(4):e37-
8.Clinical Characteristics and Treatment Outcomes in Children, Adolescents, and Young-adults with Hodgkin's Lymphoma:a KPHOG Lymphoma Working-party, Multicenter, Retrospective Study
Jae Min LEE ; Jung Yoon CHOI ; Kyung Taek HONG ; Hyoung Jin KANG ; Hee Young SHIN ; Hee Jo BAEK ; Seongkoo KIM ; Jae Wook LEE ; Nack-Gyun CHUNG ; Bin CHO ; Seok-Goo CHO ; Kyung Mi PARK ; Eu Jeen YANG ; Young Tak LIM ; Jin Kyung SUH ; Sung Han KANG ; Hyery KIM ; Kyung-Nam KOH ; Ho Joon IM ; Jong Jin SEO ; Hee Won CHO ; Hee Young JU ; Ji Won LEE ; Keon Hee YOO ; Ki Woong SUNG ; Hong Hoe KOO ; Kyung Duk PARK ; Jeong Ok HAH ; Min Kyoung KIM ; Jung Woo HAN ; Seung Min HAHN ; Chuhl Joo LYU ; Ye Jee SHIM ; Heung Sik KIM ; Young Rok DO ; Jae Won YOO ; Yeon Jung LIM ; In-Sang JEON ; Hee won CHUEH ; Sung Yong OH ; Hyoung Soo CHOI ; Jun Eun PARK ; Jun Ah LEE ; Hyeon Jin PARK ; Byung-Kiu PARK ; Soon Ki KIM ; Jae Young LIM ; Eun Sil PARK ; Sang Kyu PARK ; Eun Jin CHOI ; Young Bae CHOI ; Jong Hyung YOON ; Hoon KOOK ;
Journal of Korean Medical Science 2020;35(46):e393-
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.