PURPOSE: Despite the rapid improvement in survival rate from Burkitt lymphoma and mature B-cell lymphoblastic leukemia (B-ALL) in children, a small subset of patients do not respond to first-line chemotherapy or experience relapse (RL). Herein, we report the clinical characteristics and outcomes of these patients. MATERIALS AND METHODS: RL or refractory Burkitt lymphoma and mature B-ALL in 125 patients diagnosed from 1990 to 2009 were retrospectively analyzed. RESULTS: Nineteen patients experienced RL or progressive disease (PD). Among them, 12 patients had PD or RL less than six months after initial treatment and seven had late RL. Seven patients achieved complete response (CR), 11 had PD, and one had no more therapy. Six patients who achieved CR survived without evidence of disease and four of them underwent high-dose chemotherapy (HDC) followed by stem cell transplantation (SCT). However, 11 patients who failed to obtain CR eventually died of their disease. Five-year overall survival (OS) was 31.6+/-10.7%. OS of patients with late RL was superior to that of patients with early RL (57.1+/-18.7%, vs. 16.7+/-10.8%, p=0.014). Achievement of CR after reinduction had significant OS (p < 0.001). OS for patients who were transplanted was superior (p < 0.01). In multivariate analysis, achievement of CR after reinduction chemotherapy showed an association with improved OS (p=0.05). CONCLUSION: Late RL and chemotherapy-sensitive patients have the chance to achieve continuous CR using HDC/SCT, whereas patients who are refractory to retrieval therapy have poor prognosis. Therefore, novel salvage strategy is required for improvement of survival for this small set of patients.
Adolescent* ; B-Lymphocytes* ; Burkitt Lymphoma* ; Child* ; Drug Therapy ; Humans ; Multivariate Analysis ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prognosis ; Recurrence ; Retrospective Studies ; Stem Cell Transplantation ; Survival Rate

Novel agents to treat multiple myeloma (MM) have increased complete respone (CR) rates compared with conventional chemotherapy, and the quality of the response to treatment has been correlated with survival. The purpose of our study was to show how of early response to bortezomib combined chemotherapy influences survival in patients with newly diagnosed MM who are ineligible for stem cell transplantation. We assessed patient responses to at least four cycles of bortezomib using the International Myeloma Working Group response criteria. The endpoints were comparisons of progression free survival (PFS) and overall survival (OS) between early good response group (A group) and poor response group (B group). We retrospectively analyzed data from 129 patients registered by the Korean Multiple Myeloma Working Party, a nationwide registration of MM patients. The 3 yr PFS for the A and B groups was 55.6% and 18.4%, respectively (P < 0.001). The 3 yr OS for the A and B groups was 65.3% and 52.9%, respectively (P = 0.078). The early response to at least four cycle of bortezomib before next chemotherapy may help predict PFS in patients with MM who are ineligible stem cell transplantation.
Aged ; Antineoplastic Agents/*therapeutic use ; Boronic Acids/*therapeutic use ; Disease-Free Survival ; drugs Therapy, Combination ; Female ; Humans ; Male ; Middle Aged ; Multiple Myeloma/*drug therapy/mortality ; Predictive Value of Tests ; Pyrazines/*therapeutic use ; Registries ; Retrospective Studies ; *Stem Cell Transplantation ; Treatment Outcome

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.
Anemia ; Anemia, Diamond-Blackfan ; Aortic Coarctation ; Bone Marrow ; Congenital Abnormalities ; Diamond ; Erythrocyte Indices ; Heart Septal Defects, Ventricular ; Hemoglobins ; Hospitals, University ; Humans ; Incidence ; Korea ; Male ; Medical Records ; Registries ; Reticulocytes ; Retrospective Studies ; Steroids ; Strabismus ; Thumb ; Transplants

OBJECTIVE: This study was aimed to identify the correlation between antipsychotics-induced amenorrhea, and attitudes toward treatment and quality of life in women with schizophrenia. METHODS: Twenty female schizophrenic patients with antipsychotics-induced amenorrhea and thirty female schizophrenic patients without antipsychotics-induced amenorrhea were evaluated. Attitudes toward treatment were assessed by the Korean version of Drug Attitude Inventory (KDAI-10) and quality of life was assessed by the Korean version of World Health Organization Quality of Life Assessment Instrument-BREF (WHOQOL-BREF). The psychopathology of each patient was assessed by the Positive and Negative Syndrome Scales and the Clinical Global Impression-Severity. Adverse effects were evaluated using the Drug-Induced Extrapyramidal Symptoms Scale. Correlation analysis and multiple linear regression were conducted. RESULTS: The KDAI-10 score was not significantly correlated antipsychotics-induced amenorrhea. In WHOQOL-BREF score, social relation domain only showed significant correlation with antipsychotics-induced amenorrhea. Multiple linear regression analysis revealed that the number of family members contributed significantly to the Positive Subjective Feelings Scores of KDAI-10 and marital status contributed significantly to the social relation domain of WHOQOL-BREF in amenorrhea group. CONCLUSION: The results of present study suggest that antipsychotics-induced amenorrhea lower part of the quality of life domain in women with schizophrenia. Clinicians must pay attention to treatment of amenorrhea and various factors that correlated with attitudes toward treatment and quality of life in women with schizophrenia.
Amenorrhea ; Female ; Humans ; Linear Models ; Marital Status ; Psychopathology ; Quality of Life ; Schizophrenia ; Weights and Measures ; World Health Organization

The efficacy of tandem high-dose chemotherapy and autologous stem cell rescue (HDCT/ASCR) was investigated in patients with high-risk neuroblastoma. Patients over 1 yr of age who were newly diagnosed with stage 4 neuroblastoma from January 2000 to December 2005 were enrolled in The Korean Society of Pediatric Hematology-Oncology registry. All patients who were assigned to receive HDCT/ASCR at diagnosis were retrospectively analyzed to investigate the efficacy of single or tandem HDCT/ASCR. Seventy and 71 patients were assigned to receive single or tandem HDCT/ASCR at diagnosis. Fifty-seven and 59 patients in the single or tandem HDCT group underwent single or tandem HDCT/ASCR as scheduled. Twenty-four and 38 patients in the single or tandem HDCT group remained event free with a median follow-up of 56 (24-88) months. When the survival rate was analyzed according to intent-to-treat at diagnosis, the probability of the 5-yr event-free survival+/-95% confidence intervals was higher in the tandem HDCT group than in the single HDCT group (51.2+/-12.4% vs. 31.3+/-11.5%, P=0.030). The results of the present study demonstrate that the tandem HDCT/ASCR strategy is significantly better than the single HDCT/ASCR strategy for improved survival in the treatment of high-risk neuroblastoma patients.
Adolescent ; Child ; Child, Preschool ; Combined Modality Therapy/mortality ; Drug Therapy/*mortality ; Female ; Humans ; Infant ; Korea/epidemiology ; Longitudinal Studies ; Male ; Neuroblastoma/*mortality/*therapy ; Prevalence ; Risk Assessment/methods ; Risk Factors ; Stem Cell Transplantation/*mortality ; Survival Analysis ; Survival Rate ; Treatment Outcome

PURPOSE: To evaluate the efficacy of primary chemotherapy combined with local therapy in the treatment of retinoblastomas not treatable with a single therapeutic method. METHODS: We performed a retrospective chart review of 227 patients diagnosed with retinoblastoma. Sixty-five eyes in 52 patients had tumors not treatable with a single therapeutic method and received primary chemotherapy combined with local therapy as needed. RESULTS: Tumor control and eye salvage was achieved in 34 of the 65 eyes; the probability of ocular survival was 46.56% using the Kaplan-Meier method. Forty-three of the 65 eyes were group D or E tumors, in which tumor control and eye salvage was achieved in 16 eyes. Twenty eyes were treated with chemotherapy only, while 28 eyes received one additional modality of local therapy, and 17 eyes received two modalities of local therapy. Of the eyes treated with chemotherapy only, tumor control was achieved in 5 eyes. CONCLUSIONS: Primary chemotherapy combined with local therapy can be effective and safe in the treatment of retinoblastomas otherwise untreatable with other therapeutic methods, such as group D and E retinoblastomas. More vigorous treatment with more local therapeutic methods combined may yield even better results.
Antineoplastic Agents/*therapeutic use ; Child, Preschool ; Cryotherapy/*methods ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Hyperthermia, Induced/*methods ; Infant ; Infant, Newborn ; Laser Coagulation/*methods ; Male ; Ophthalmoscopy ; Retinal Neoplasms/diagnosis/physiopathology/*therapy ; Retinoblastoma/diagnosis/physiopathology/*therapy ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome ; *Visual Acuity

BACKGROUND: Leukemic cells originate from hypoxic bone marrow, which protects them from anti-cancer drugs. Although many factors that cause drug resistance in leukemic cells have been studied, the effect of hypoxia on drug-induced apoptosis is still poorly understood. METHODS: In this study, we examined the effect of hypoxia on anti-leukemic drug resistance in leukemic cell lines treated with cobalt chloride (CoCl2), a hypoxia-mimetic agent. Cellular proliferation was evaluated using the methyl thiazolyl tetrazolium (MTT) assay. Flow cytometry analysis and western blots were performed to investigate apoptosis-related proteins. RESULTS: Unlike its previously known apoptotic effect, the expression of HIF-1alpha increased the survival rate of human promyelocytic leukemia HL-60 cells when these cells were exposed to anti-leukemic drugs; these effects were mediated by heat-shock protein HSP70 and the pro-apoptotic protein Bax. CONCLUSION: These findings may provide new insights for understanding the mechanisms underlying hypoxia and for designing new therapeutic strategies for acute myeloid leukemia.
Anoxia ; Apoptosis ; Arsenicals ; Blotting, Western ; Bone Marrow ; Cell Line ; Cell Proliferation ; Cobalt ; Drug Resistance ; Flow Cytometry ; Heat-Shock Proteins ; HL-60 Cells ; Humans ; Leukemia ; Leukemia, Myeloid, Acute ; Oxides ; Proteins ; Survival Rate

BACKGROUND: Cytogenetic abnormalities (CAs) have been reported frequently in patients with otherwise typical aplastic anemia (AA), but their implications in the prognosis and in the evolution to hematologic malignancies are controversial. METHODS: We retrospectively analyzed 127 adult AA patients who had successful cytogenetic analysis at initial diagnosis. RESULTS: The patients were classified into 3 groups according to the initial and follow-up results of cytogenetic profiles. Group 1 included patients who had persistent AA with normal cytogenetic profiles (N=117); Group 2, those who had a normal cytogenetic profile at initial diagnosis but later acquired CA (N=4, 3.1%); and Group 3, those who had CA at the initial diagnosis, regardless of follow-up cytogenetic status (N=6,4.7%). In Group 2, 2 patients later developed CA without progression to acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS); the other 2 patients later progressed to AML. None of the patients in Group 3 progressed to AML or MDS. There was no significant difference in overall survival between Groups 1 and 3. CONCLUSION: AA patients with CA at initial diagnosis or follow-up may not be at greater risk for evolution to AML or MDS, or show shorter survival periods. Prospective studies and a larger patient samples are needed to establish the clinical relevance of CA.
Adult ; Anemia, Aplastic ; Chromosome Aberrations ; Cytogenetic Analysis ; Cytogenetics ; Follow-Up Studies ; Hematologic Neoplasms ; Humans ; Incidence ; Leukemia, Myeloid, Acute ; Myelodysplastic Syndromes ; Prognosis ; Retrospective Studies

Acquired hemophilia is a rare but potentially life-threatening hemorrhagic disorder caused by the development of autoantibodies against coagulation factor VIII. Concentrates of human factor VIII, desmopressin, activated prothrombin complex concentrates, recombinant activated factor VII can all be used to control episodes of acute bleeding. The recent availability of bypassing agents like recombinant activated factor VII has been shown to be clinically safe and effective as treatment for acute bleeding. In this case report, a 67 year-old male patient with Rh negative blood type developed gross hematuria and bleeding after transurethral resection due to prostatic hypertrophy. After vesicocutaneous fistular reduction operation, post-operative bleeding was presented. The acute bleeding was controlled successfully by the combined treatment with recombinant activated factor VII (Novo seven(R)) and prednisone.
Autoantibodies ; Blood Coagulation Factors ; Deamino Arginine Vasopressin ; Factor VIIa ; Factor VIII ; Hematuria ; Hemophilia A ; Hemorrhage ; Hemorrhagic Disorders ; Humans ; Male ; Prostatic Hyperplasia ; Prothrombin

Behcet's disease is a relapsing inflammatory disorder characterized by vasculitis of unknown cause and has been reported rarely in association with malignant diseases. In most cases the autoimmune nature of Behcet's disease and the long-term immunosuppressive therapy for disease control have been proposed to be responsible for malignant transformation. Although a few cases of various solid tumor and myelodysplastic syndrome have been reported in association with Behcet's disease, acute leukemia has seldom been associated with Beh?et's disease in Korea. We report a case of 38-year-old man with acute myeloblastic leukemia association with a Behcet's disease who had not received long-term treatment.
Acute Disease ; Adult ; Humans ; Korea ; Leukemia ; Leukemia, Myeloid, Acute ; Myelodysplastic Syndromes ; Vasculitis