Journal Detail Information

1

Cite

Share

Mixed Mononuclear Cell Culture of Cord Blood and Adult Peripheral Blood.

Korean Journal of Pediatric Hematology-Oncology.2002;9(2):211-219.

PURPOSE: Umbilical cord blood as the source of hematopoietic stem cells has several advantages over bone marrow cells for transplant purpose, but the limitation inherent in the cord blood is its limited number of hematopoietic stem cells. The responses of cord blood mononuclear cells (MNC) which were stimulated by unrelated cord blood MNC or adult peripheral blood MNC to investigate the possibility of unrelated, HLA-mismatched multiple cord blood transplantation were evaluated. METHODS: Nineteen cord blood and 7 adult peripheral blood samples were used. Each of the MNC from cord blood and adult peripheral blood was cultured before and after 2,000 cGy irradiation. Mixed MNC cultures with cord blood and irradiated different cord blood or irradiated adult peripheral blood were performed. RESULTS: The mean percentages of MNC counts compared to day 0 of day 2 and day 7 cultures of mixed MNC cultures with cord blood and irradiated different cord blood were 92.5 55.8%, 57.4 51.0% and those of mixed MNC cultures with cord blood and irradiated adult peripheral blood were 143.4 132.7%, 113.4 95.1%, respectively. The difference of the cell count changes in 2 types of MNC cultures was statistically significant (P<0.05). CONCLUSION: The proliferative responses of cord blood MNC to different cord blood MNC were significantly lower than those to adult peripheral blood MNC. The study for the investigation of clinical applicability of cord blood transplantation from multiple unrelated donors to overcome the limitation of the cell dose of cord blood transplantation and the shortage of related or unrelated HLA-matched donors is needed.
Adult* ; Bone Marrow Cells ; Cell Count ; Cell Culture Techniques* ; Fetal Blood* ; Hematopoietic Stem Cells ; Humans ; Tissue Donors ; Unrelated Donors

2

Cite

Share

A Comparative Analysis between Flow Cytometry and RT-PCR Methods for the Detection of Minimal Residual Disease in Neuroblastoma.

Byoung Chul KWON ; Kyung Sik KIM ; Sung Chul WON ; Seung Hwan OH ; Chuhl Joo LYU ; Chang Hyun YANG

Korean Journal of Pediatric Hematology-Oncology.2002;9(2):203-210.

PURPOSE: A sensitive assay to detect minimal residual disease in neuroblastoma is necessary for accurate assessment of disease status and optimal treatment. In this study, we compared the usefulness of sensitive methods, flow cytometry and RT-PCR for the detection of minimal residual disease in neuroblastoma. METHODS: Eighteen patients who were newly diagnosed and treated at Severance Hospital since 1999 were included in this study. Samples from bone marrow, peripheral blood, and peripheral blood stem cell product were examined for tumor cell contamination by RT-PCR (TH RT-PCR) to detect tyrosine hydroxylase mRNA and by flow cytometry identifying CD9+/CD56+/CD45- cells. RESULTS: We analyzed 20 cases from 18 patients, which were assayed by both methods at the same time. Among 20 cases, 16 cases showed same results, which were compatible with histologic results and clinical course, and 4 cases showed different results. One case of them showed positive result in histology and flow cytometry, but negative result in TH RT-PCR. The other 3 cases showed negative results in flow cytometry, but positive results in TH RT-PCR, and 1 patient of them relapsed. Among 16 patients, 2 patients, showing positive results in only TH RT-PCR, relapsed. CONCLUSION: Detection of minimal residual disease using TH RT-PCR and flow cytometry was effective and useful in evaluating disease status and deciding for proper treatment.
Bone Marrow ; Flow Cytometry* ; Humans ; Neoplasm, Residual* ; Neuroblastoma* ; RNA, Messenger ; Stem Cells ; Tyrosine 3-Monooxygenase

3

Cite

Share

Treatment with IDA-FLAG (idarubicin, fludarabine, cytarabine, G-CSF) Regimen in Children with Myelodysplastic Syndrome and Relapsed/Refractory Acute Myelogenous Leukemia.

Sang Hee LEE ; Sun Young KIM ; Pil Sang JANG ; Nak Gyun CHUNG ; Bin CHO ; Dae Chul JEONG ; Hack Ki KIM

Korean Journal of Pediatric Hematology-Oncology.2002;9(2):193-202.

PURPOSE: The prognosis of refractory or relapsed acute myelogenous leukemia (AML) is poor and effective reinduction regimens are rare. Myelodysplastic syndrome (MDS) is a heterogenous group of clonal disorders of hematopoiesis and the outcome for children with MDS is poor, and the optimal treatment of MDS has not been defined. This study was undertaken to investigate the therapeutic results of IDA-FLAG (idarubicin, fludarabine, cytarabine, G-CSF) in children with MDS and relapsed or refractory AML. METHODS: Eleven children with refractory or relapsed AML and ten with MDS were treated with the IDA-FLAG regimen, a combination therapy of idaraubicin (day 1~3, 12 mg/m2/day), fludarabine (day 1~5, 30 mg/m2/day), cytarabine (day 1~5, 2 g/m2/day) and G-CSF (day 0~5, 400 g/m2/day & day 12 up to ANC > 1,000/L, 5 g/kg/day). RESULTS: In AML group, they received a total 15 courses of IDA-FLAG. Complete remission (CR) was achieved in 4/11 (36.4%) with median remission duration of 3 (2~6) months. In MDS group, they received 13 courses of IDA-FLAG and 8 courses of FLAG (IDA-FLAG without idarubicin). CR was achieved in 6/8 (75.0%) with median remission duration of 4 (2~13) months. All the patients experienced grade 4 hematologic toxicities. The median duration of neutropenia (<500/ mm3) and thrombocytopenia (<30,000/mm3) was 23 days (20~46 days) and 23.5 days (16~44 days) in AML, meanwhile those were 18 days (14~30 days) and 16 days (13~32 days) in MDS, respectively. Infectious complications were the main non-hematological toxicity. Two patients died of sepsis and intracerebral hemorrhage on day 7 and 27, respectively. CONCLUSION: IDA-FLAG may be an efficient reinduction therapy for resistant and intensively pretreated AML and an effective regimen for poor prognostic MDS with acceptable toxicity, even though remission duration seems to be relatively short. Therefore, an intensified post-remission therapy seems necessary.
Cerebral Hemorrhage ; Child* ; Cytarabine* ; Granulocyte Colony-Stimulating Factor ; Hematopoiesis ; Humans ; Leukemia, Myeloid, Acute* ; Myelodysplastic Syndromes* ; Neutropenia ; Prognosis ; Sepsis ; Thrombocytopenia

4

Cite

Share

The Clinical Features and Prognosis of Leukemia in Down Syndrome.

Ye Na CHOI ; Jee Hyun CHUN ; Seung Hwan OH ; Chuhl Joo LYU ; Chang Hyun YANG ; Kir Young KIM

Korean Journal of Pediatric Hematology-Oncology.2002;9(2):186-192.

PURPOSE: There are several reports that the risk of development of leukemias is much higher in Down syndrome (DS) children than in non DS children. But there are a few reports about the clinical features of leukemia in Down syndrome and the prognosis in Korea. The object of this study is to evaluate clinical features, treatment results and the prognosis of leukemia of Down syndrome patients. METHODS: We conducted retrospective reviews in 10 children with leukemia of Down syndrome who were admitted to the Department of Pediatrics in Yonsei University Hospital between March 1986 and December 2000. We analyzed the clinical features, laboratory findings and survival rates. RESULTS: A male to female ratio was 1:1.25. Median age at diagnosis was 2 years 8 months. Initial symptoms were hepatosplenomegaly, petechiae, fever and upper respiratory infection symptoms. The number of patients by the type was as followed:acute myeloid leukemia (AML) 7 (70%), acute lymphocytic leukemia 2 (20%), acute mixed lineage leukemia 1 (10%). There were 4 cases of M7 subtype in AML. The median peripheral blood cell counts were as followed; leukocyte was 41,000/muL, hemoglobin was 8.7 g/dL, the platelet was 103,000/muL. The five years event free survival rate after diagnosis was 87.5% (7/8). The one patient relapsed and another one patient died of cardiac anomaly. CONCLUSION: There seemed to be several differences of clinical features between DS leukemia and non DS leukemia, especially prognosis. Multi-centered well organized study should be done to confirm our observation.
Blood Cell Count ; Blood Platelets ; Child ; Chromosomes, Human, Pair 21 ; Diagnosis ; Disease-Free Survival ; Down Syndrome* ; Female ; Fever ; Humans ; Korea ; Leukemia* ; Leukemia, Myeloid ; Leukemia, Myeloid, Acute ; Leukocytes ; Male ; Pediatrics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis* ; Purpura ; Retrospective Studies ; Survival Rate

5

Cite

Share

Detection of Minimal Residual Disease Using Real-Time Quantitative Polymerase Chain Reaction in Children with Philadelphia Chromosome Positive Acute Lymphoblastic Leukemia.

Sangrhim CHOI ; Bin CHO ; Sun Young KIM ; Pil Sang JANG ; Nak Gyun CHUNG ; Dae Chul JEONG ; Hack Ki KIM

Korean Journal of Pediatric Hematology-Oncology.2002;9(2):177-185.

PURPOSE: The aim of this study was to detect the bcr-abl/abl mRNA ratio of the patients with Philadelphia chromosome positive childhood acute lymphoblastic leukemia (ALL) after completing chemotherapy or bone marrow transplantation (BMT), by the real-time quantitative polymerase chain reaction (RQ-PCR). It was also to be proved that these quantitative changes of minimal residual disease (MRD) can be used for early detection of relapse and for treatment response. METHODS: The subjectives of this study were 8 patients diagnosed with Philadelphia chromosome positive childhood acute lymphoblastic leukemia (ALL) from January to November in the year 2001. The change of bcr/abl transcript with chemotherapy were evaluated in 6 patients out of them. RESULTS: All of 6 patients reached in hematologically complete remission state by the induction chemotherapy, but the MRD was cytogenetically detected in 5 out of the 6 patients. While the 5 patients were in the hematologically remission state, only 1 patient had the cytogenetical remission after the serial consolidation chemotherapy at the maximum of 6 times. The increased amount of bcr-abl/abl mRNA ratio (up to 10 3) were correlated with the positive real time polymerase chain reaction (RT-PCR) result, but the results of bcr/abl fluorescent in situ hybridization (FISH) were not correlated with the results of RT-PCR and bcr-abl/abl mRNA ratio. The change of bcr/abl transcript was evaluated in 4 patients after BMT. The RT-PCR in 1 month after BMT showed the negative result in all 4 patients in our study. The bcr-abl/abl mRNA ratio in all patients was constantly decreased. However, bcr-abl/abl mRNA ratio in one patient was increased in 6 months after BMT, and the results of second RT-PCR showed positive value, which made the cyclosporine be discontinued immediately. In the 9th month following BMT, the ratio of bcr-abl/abl mRNA was abruptly decreased and the results of second RT-PCR showed the negative value. CONCLUSION: We suggest that RT-PCR or RQ-PCR rather than FISH is more sensitive and effective for detection of MRD. We also observed that Philadelphia chromosome positive childhood ALL could be cured with the BMT, not with the chemotherapy alone, by monitoring MRD.
Bone Marrow Transplantation ; Child* ; Consolidation Chemotherapy ; Cyclosporine ; Drug Therapy ; Humans ; In Situ Hybridization, Fluorescence ; Induction Chemotherapy ; Neoplasm, Residual* ; Philadelphia Chromosome* ; Polymerase Chain Reaction* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Real-Time Polymerase Chain Reaction ; Recurrence ; RNA, Messenger

6

Cite

Share

Treatment of B-cell Acute Lymphoblastic Leukemia and B-cell Lymphoma.

Eun Sil PARK ; Sang Duk KIM ; Hyoung Jin KANG ; Hyoung Soo CHOI ; Hee Young SHIN ; Hyo Seop AHN

Korean Journal of Pediatric Hematology-Oncology.2002;9(2):166-176.

PURPOSE: We report here the improved survival rate of B-cell acute lymphoblstic leukemia (B-ALL) and B-cell non-Hodgkin's lymphoma (B-NHL) treated with a short, intensive multiagent chemotherapy and the treatment related toxicities and complications. METHODS: From Oct. 1997 to Apr. 2002, 10 patients were enrolled. Patients were classified into three groups (Group A, B, C) according to tumor burden and were treated with CCG 5961, UKCCSG 9600, and LMB96 protocol. Induction chemotherapy included cyclophsophamide, vincristine, prednisolone, doxorubicin and high dose (HD) methotrexate (COPADM). Consolidation chemotherapy included HD methotrexate, HD cytarabine and etoposide (CYM; group B, CYVE; group C). In one patient, HD chemotherapy with stem cell rescue was used because residual disease was detected after consolidation chemotheapy. RESULTS: Four patients were B-ALL and six patients were B-NHL (A; 1, B; 2, C; 7). Regimen was changed in 1 patient because of residual disease (B-- B-Lymphocytes* ; Bone Marrow ; Consolidation Chemotherapy ; Cytarabine ; Doxorubicin ; Drug Therapy ; Etoposide ; Fever ; Follow-Up Studies ; Humans ; Induction Chemotherapy ; Leukemia ; Lymphoma, B-Cell* ; Lymphoma, Non-Hodgkin ; Methotrexate ; Neutropenia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prednisolone ; Recurrence ; Renal Dialysis ; Stem Cells ; Stomatitis ; Survival Rate ; Tumor Burden ; Tumor Lysis Syndrome ; Vincristine

7

Cite

Share

Allogeneic Hematopoietic Stem Cell Transplantation in Children with Acute Lymphoblastic Leukemia: Experiences in a Single Center.

Ki Woong SUNG ; Keon Hee YOO ; Eun Hee CHUNG ; Chang Kyu KANG ; Bong Lim KIM ; Kwang Bin MOON ; Jong Hee HWANG ; Hong Hoe KOO ; Do Hoon LIM ; Quehn PARK ; Sun Hee KIM ; Dae Won KIM ; Hye Kyung PARK

Korean Journal of Pediatric Hematology-Oncology.2002;9(2):155-165.

PURPOSE: To improve survival of children with acute lymphoblastic leukemia (ALL), allogeneic hematopoietc stem cell transplantation (HSCT) was applied. METHODS: From June 1999 to May 2002, 27 children with ALL received allogeneic HSCT at Samsung Medical Center. Patients in complete remission (CR) who received HLA-matched HSCT before relapse when HSCT was indicated were assigned to standard-risk, otherwise were assigned to high-risk. Cyclophosphamide and total body irradiation was basic conditioning regimen. For prophylaxis of GVHD, cyclosporine alone in related HSCT and cyclosporine methotrexate methylprednisolone in unrelated HSCT were used. RESULTS: Fifteen patients in first CR including 6 induction failures, 3 MLL rearrangements and 2 Philadelphia chromosomes, and 3 patients in second CR were assigned to standard-risk. Thirteen related HLA-matched, 11 unrelated HLA-matched, 2 related HLA-mismatched and 1 unrelated HLA-mismatched HSCT were applied. Sixteen of 18 standard-risk patients are still alive with median follow-up of 12.5 (range: 2~37) months and 13 of them are disease-free without relapse. Event-free survival rate (EFS) in 18 standard-risk and 9 high-risk patients were 68.2% and 14.8%, respectively. Confined to standard-risk patients, EFS in related and unrelated HSCT were 75.0%, 60.0%, respectively. CONCLUSION: When allogeneic HSCT is indicated in childhood ALL with available HLA-matched donor, early transplantation before clinical aggravation seems to be necessary.
Child ; Cyclophosphamide ; Cyclosporine ; Disease-Free Survival ; Follow-Up Studies ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Methotrexate ; Methylprednisolone ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Recurrence ; Stem Cell Transplantation ; Tissue Donors ; Whole-Body Irradiation

8

Cite

Share

A Case of Langerhans Cell Histiocytosis with Hypothalamic Mass.

Woe Sook YOON ; Ji Hae KANG ; Yoo Rah HONG ; Jae Sun PARK ; Bong Kwon CHUN ; Mi Hee JUNG ; Chang Woo MOON

Korean Journal of Pediatric Hematology-Oncology.2005;12(1):131-137.

A 14-month-old girl presented with petechial skin lesions and polydipsia was diagnosed as Langerhans cell histiocytosis (LCH) and responded fairly well to multiple chemotherapies using vincristine, cyclophosphamide, and prednisone. 3 years later, relapses were more common with short periods of remissions in spite of using more intensive therapy with vinblatine and etoposide. At age of 4.5, sudden weight gain and abnormal behavior led to MRI study and revealed an hypothalamic mass. Radiation of 1, 800 cGy was given to the mass and followed by a 75% decrease in measuring and remission of the obesity. Although, there was no evidence of tumor progression in the hypothalamus, she died of sepsis due to systemic progression of the disease at age of 5. LCH commonly present with the symptoms of diabetes insipidus, but hypothalamic mass is not common. We report this case with a brief review of literatures.
Cyclophosphamide ; Diabetes Insipidus ; Drug Therapy ; Etoposide ; Female ; Histiocytosis, Langerhans-Cell* ; Humans ; Hypothalamus ; Infant ; Magnetic Resonance Imaging ; Obesity ; Polydipsia ; Prednisone ; Recurrence ; Sepsis ; Skin ; Vincristine ; Weight Gain

9

Cite

Share

A Case of Incidentaloma Compromising of Pheochromocytoma and Adrenocortical Adenoma in an Infant.

Ji Hea KANG ; Woe Sook YOON ; Jae Sun PARK ; Jong Cheol KIM ; Young Ok KIM ; Won Duk KIM

Korean Journal of Pediatric Hematology-Oncology.2005;12(1):125-130.

Pheochromacytoma, although occasionally present with adrenal cortical hyperfunction, is rarely associated with nonfunctioning adrenal cortical tumor. To our knowledge, eight cases of phemchromocytoma associated with adrenocortical adenoma have been reported in the literature, including a case in a Korean adult female. An adrenal mass is considered an incidentaloma when there is no history or physical findings suggesting an adrenal functional disorder or tumor. The majority of adrenal masses are nonfunctioning adrenocortical adenomas. In our case, left adrenal mass was found incidentally by ultrasonography after birth, done because of mother's oligohydroamnios. Abdomial CT study revealed an adrenal tumor, and a surgical resection was performed. The specimen showed a coincident pheochromocytoma and adrenocortical adenoma. The patient seems to be the first case of coexistence of nonfunctioning pheochromocytoma and adrenocortical adenoma in one adrenal tumor. We report this case with the review of literatures.
Adrenocortical Adenoma* ; Adult ; Female ; Humans ; Infant* ; Parturition ; Pheochromocytoma* ; Ultrasonography

10

Cite

Share

A Case of Congenital Hepatoblastoma.

Min Jung SUNG ; Jae Hong PARK ; Hae Young KIM ; Young Tak LIM

Korean Journal of Pediatric Hematology-Oncology.2005;12(1):120-124.

Hepatoblastoma is the most common primary malignant hepatic tumor in infancy and childhood. But congenital hepatoblastomas are extremely rare and show distinctive, but important features when compared with tumors diagnosed in children beyond the neonatal age. They have different clinical presentations, higher incidence of pure fetal histology, significant risk for systemic metastasis, and worse outcome. The treatment of congenital hepatoblastoma should be the same as in older children, i.e., primary chemotherapy with delayed resection. We report a case of congenital hepatoblastoma in a 29-day-old boy who was known to have a well-defined ovoid hypoechoic mass at liver demonstrated by fetal sonography.
Child ; Drug Therapy ; Hepatoblastoma* ; Humans ; Incidence ; Liver ; Male ; Neoplasm Metastasis

DISPLAY OPTIONS

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share