Myelodysplastic syndrome (MDS) is a clonal stem cell disorder characterized by ineffective hematopoiesis, multilineage dysplasia, peripheral cytopenias with normocellular or hypercellular marrow, and susceptibility to leukemic transformation. MDS represents a heterogenous group of disorders with a wide spectrum of clinical, morphological, biologic, and genetic characteristics. MDS is classified according to the World Health Organization criteria and the International Prognostic Scoring System. Risk-adapted treatment strategies have been developed in consideration of the advanced age of patients and to improve the clinical course of the disease. The pathophysiology, cytogenetic/molecular profiles, clinical characteristics and treatment modalities according to the prognostic groups will be described. In the future, a combination of treatment modalities to increase gene reactivation and to take advantage of increased expression of target genes may be critical to improve clinical outcomes. Multiple pathways may be involved in the MDS phenotype, and combination therapies, including novel agents, may be required to make further progresses in the treatment of this disease.
Bone Marrow ; Classification ; Diagnosis ; Hematopoiesis ; Humans ; Myelodysplastic Syndromes* ; Phenotype ; Stem Cells ; World Health Organization

No abstract available.
Stomach Neoplasms* ; Stomach* ; Water*

Acute myeloid leukemia (AML) is a representative blood cancer, accounting for most adult leukemia cases in Korea. Until recently, intensive chemotherapy and hematopoietic stem cell transplantation were the only curative treatment options for AML. However, the recent introduction of new drugs is bringing about changes in the strategic paradigm for the treatment of AML.Current Concepts: Along with the clinical eligibility for receiving intensive treatment and hematopoietic stem cell transplantation, the most critical determinants in treating AML are precise classification and risk stratification based on cytogenetic and molecular information. The recently revised World Health Organization classification, newly proposed International Consensus Classification, and the latest version of the European LeukemiaNet risk stratification reflect the importance of cytogenetic and molecular information. Although there have been no significant changes for a long time in the landscape of AML, especially in the field of treatment, the treatment paradigm has started to evolve with the introduction of new drugs. This evolution is led by FLT3 inhibitors, Bcl-2 inhibitors, isocitrate dehydrogenase inhibitors, target agents against CD33 antigens, and liposomal formulations of chemotherapeutics.Discussion and Conclusion: Successful initial treatment to induce complete remission followed by post-remission treatment to remove residual disease can lead to the achievement of long-term survival and cure goals in AML. We hope that new drugs will markedly improve the treatment outcomes for patients with AML.

BACKGROUND: Cerebrospinal fluid (CSF) examination can be used to verify the presence of primary malignancies as well as cases of central nervous system (CNS) metastasis. Because of its importance, there have been several studies concerning the sensitivity of CSF cytology. To determine the practical use and reproducibility of diagnoses based on CSF cytology, we evaluated this test by analyzing cytology results from consecutive CSF samples. METHODS: Between July 2010 and June 2013, 385 CSF cytology samples from 42 patients were collected. The samples were gathered using a ventricular catheter and reservoir. CSF cytology of all patients was examined more than two times with immunocytochemistry for cytokeratin. RESULTS: Primary neoplastic sites and histologic types of patients' metastatic cancer were diverse. The overall sensitivity for detecting malignancy was 41.3%. Even within short-term intervals, diagnoses frequently changed. CONCLUSIONS: Our results were inconsistent, with low sensitivity, when compared to the results of previous studies. However, CSF evaluation can still provide valuable diagnostic and prognostic information because adjuvant treatments are now routinely performed in patients with CNS metastasis. Negative CSF cytology results should not be ignored, and continuous CSF follow-up is essential for following the clinical course of patients with metastatic cancer involving the CNS.
Catheters ; Central Nervous System ; Cerebrospinal Fluid* ; Diagnosis ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Keratins ; Neoplasm Metastasis

BACKGROUND: Hemolytic disease of the fetus and newborn (HDFN) is a condition in which immune hemolytic anemia occurs in fetuses or newborns as a result of maternal alloimunized antibodies transfer. Antibody elution test and direct antiglobulin test (DAT) can be performed to diagnose HDFN; maternal originated antibodies cannot be confirmed if DAT is utilized alone. In this study, we analyzed the clinical significance of implementing concurrent DAT and antibody elution test in diagnosing HDFN. METHODS: We retrospectively analyzed the DATs and antibody elution tests that were simultaneously conducted in a period of 11 years, between 2005 and 2015, in newborns that received hemoglobin, reticulocyte, and total bilirubin tests. According to the results of these tests, the number of newborns diagnosed with HDFN was measured. Furthermore, the sensitivity and specificity of DAT and antibody elution test were compared. RESULTS: Among 325 newborns, the results of DATs and antibody elution tests were both negative in 208 (64.0%), negative and positive, respectively, in 80 (24.6%), positive and negative in 10 (3.1%), both positive in 27 (8.3%). When this was compared to the clinical diagnosis of HDFN, more sensitive and specific diagnoses were possible when implementing DAT and antibody elution test together (sensitivity of 76.9% for antibody elution test and specificity of 90.3% for DAT). Twenty-six (8.0%) newborns suspected for HDFN showed clinically significant hemolytic anemia. CONCLUSION: It is necessary to conduct both DAT and antibody elution test when HDFN is suspected. The severity of hemolysis in HDFN can be indirectly anticipated using an antibody elution test confirming maternal originated alloantibodies.
Anemia, Hemolytic ; Antibodies ; Bilirubin ; Coombs Test* ; Diagnosis ; Fetus* ; Hemolysis ; Humans ; Infant, Newborn* ; Isoantibodies ; Reticulocytes ; Retrospective Studies ; Sensitivity and Specificity

BACKGROUND: In patients who had serum autoantibody that reacted with all screening red blood cells (panagglutination), waiting for compatible blood is likely to delay a needed transfusion. In some cases of severely diminished hemoglobin counts, the least incompatible blood may be transfused. However, the least incompatible transfusion therapy is challenged by the presence of unexpected antibody in patient's serum, which may cause a transfusion reaction. The aim of this study was to evaluate the effect of the least incompatible transfusion on clinical outcomes in patients with panagglutination. METHODS: We conducted a retrospective study of 49 patients with panagglutination on an unexpected antibody screening test between January 2006 and July 2010. In 36 patients having the least incompatible blood transfusion, changes in hemoglobin and lactate dehydrogenase (LD) values before and after transfusion were analyzed. One year mortality after initial need for transfusion was documented. RESULTS: In all 36 patients who underwent transfusion, hemoglobin values showed an increase of 1.2 (0.0~3.0) g/dL per unit without occurrence of acute transfusion reactions indicated by an increase in the LD level. The least incompatible transfusion did not show an association with increased all-cause mortality. CONCLUSION: As an alternative to the time consuming process of alloantibody detection, patients with severe anemia can be effectively transfused with "least incompatible units" in an emergency clinical setting without experiencing acute transfusion reactions.
Anemia ; Blood Group Incompatibility ; Blood Transfusion ; Emergencies ; Erythrocytes ; Hemoglobins ; Humans ; L-Lactate Dehydrogenase ; Mass Screening ; Retrospective Studies

Curschmann's spirals morphologically similar to those seen in sputum were found in cervico-vaginal smears of six patients ranged from 28 to 40 years of age, during 18 months from January. 1985 to June, 1986. The prevalence was 1 in 2147 smears in that period. All of them had gynecologic disorders without systemic effect such as chronic cervictis in three, leiomyoma in one, pelvic inflammatory disorder in one, and primary infertility in one. The Curshmann's spirals in the smear showed varying degrees of maturation from wavy incipient ones to highly coiled mature ones, admixed with thick mucinous back- ground, suggesting of their production in the uterine cervix itself. Also the recent history of undergone cryocautery, electrocautery or parturition suggest its production in the endocervical gland due to mechanical obstruction and/or change in biochemical composition of mucus.
Cervix Uteri ; Diabetes Insipidus* ; Electrocoagulation ; Female ; Humans ; Infertility ; Leiomyoma ; Mucins ; Mucus ; Parturition ; Pedigree* ; Pilomatrixoma ; Prevalence ; Receptors, Vasopressin* ; Sputum ; Vasopressins*

This study aimed to analyze the overall survival period of adult lymphoblastic lymphoma patients treated with various therapeutic regimens, and to assess the determinants affecting survival outcome. Twenty-five adult patients with lymphoblastic lymphoma who had been treated at Severance Hospital, Yonsei University College of Medicine, Seoul, Korea from June 1996 to June 2005 were analyzed retrospectively. As an initial remission induction chemotherapy, the hyper-CVAD regimen was performed in eight patients, the Stanford/Northern California Oncology Group (NCOG) regimen in five, the CAVOP regimen in four, the m-BACOP regimen in three, and the CHOP regimen in one patient. Patients were divided into two groups according to their therapeutic modalities. Twenty patients received conventional chemotherapy alone and five received subsequent PBSCT after conventional chemotherapy. Four patients of the PBSCT group underwent autologous PBSCT and one underwent allogeneic PBSCT. The overall response rate was 80% (60% showing a complete response, 20% showing a partial response) and the relapse rate was 73.3%. The overall survival (OS) rate was 55.1% at 1 year, 31.5% at 5 years, and 23.6% at 9 years. The disease-free survival (DFS) rate was 46.7% at 1 year and 30.0% at 7 years. The 5-year OS rate in relation to the regimens was 60% with the Stanford/NCOG regimen, 50% with the CAVOP regimen, and 33.3% with the m-BACOP regimen. The patients treated with the hyper-CVAD regimen had an 18.2% 2-year OS rate, and other patients with CHOP or COPBLAM-V expired early in their course. The OS rate in patients treated with conventional chemotherapy alone was 19.8%, whereas patients treated with subsequent PBSCT after chemotherapy showed 50% overall survival (p=0.25). The age at presentation influenced the outcome of the patients (p=0.01). The Stanford/NCOG regimen is an effective initial choice of therapy for lymphoblastic lymphoma patients, and is superior to the hyper-CVAD regimen in complete response rate and overall survival rate (p=0.36). Addition of PBSCT after chemotherapy may be needed for achieving optimal outcomes.
Treatment Outcome ; Time Factors ; Stem Cell Transplantation/methods ; Retrospective Studies ; Prognosis ; Middle Aged ; Male ; Lymphoma, Lymphoblastic/*mortality/*therapy ; Humans ; Female ; Disease-Free Survival ; Antineoplastic Agents/pharmacology ; Aged ; Adult ; Adolescent

Hodgkin's disease (HD) is a hematologic malignancy which shows common features regardless of race, but racial differences may be considered with certain clinical characteritcs. HD in Korea shows somewhat different characteristics when compared to cases in Western countries. We evaluated the clinical and histopathologic characteristics of HD, the outcomes of various chemotherapy regimens, and prognostic factors of HD in Korea. One hundred and five patients with initial histopathologic diagnosis of Hodgkin's disease were retrospectively reviewed 20 years after diagnosis at Yonsei University College of Medicine. Nodular sclerosis was the most common histopathogic subtype (41%) and mixed cellularity was nearly as common (40%). The overall complete remission rate (CR) was 87.6%. The disease-free survival (DFS) and overall survival (OS) rate were 79.2% and 84.8% at 5-years, 70% and 79.2% at 10- and 20-years. There were no significant differences in CR rate and DFS, but OS rates were significantly higher in m-BACOP and ABVD regimen. Univariate analysis revealed that age, B-symptom, ECOG scale, Ann Arbor stage, international prognostic index, and serum beta2-microglobulin level were significant prognostic factors for both DFS and OS. Multivariate analysis demonstrated that age, B symptoms, and ECOG scale were significant prognostic factors for OS only. In conclusion, the survival rates of HD patients in our center were superior to those of previous reports in Korea and Western countries. Considering the higher OS rate and decreased incidence of side effects, the ABVD regimen may be recommended for the initial treatment of Hodgkin's disease.
Treatment Outcome ; Remission Induction ; Prognosis ; Middle Aged ; Male ; Korea ; Humans ; Hodgkin Disease/mortality/*therapy ; Follow-Up Studies ; Female ; Disease-Free Survival ; Child, Preschool ; Child ; Antineoplastic Agents/*pharmacology ; Aged, 80 and over ; Aged ; Adult ; Adolescent

PURPOSE: Follicular lymphoma (FL) is an indolent non-Hodgkin's lymphoma that is highly sensitive to radiotherapy (RT). However, the effectiveness of RT has not been well established. We reviewed our experiences to assess the role of RT for FL and analyze treatment results. MATERIALS AND METHODS: Retrospective analysis was done on 29 patients who received first RT between January 2003 and August 2013. Of 23 early stage (stage I, II) patients, 16 received RT alone, four received chemotherapy followed by RT, two received RT postoperatively, and one received salvage RT for relapse after resection. Six advanced-stage (stage III, IV) patients received RT after chemotherapy: two received consolidation RT, three received salvage RT for residual lesions, and one received RT for progressive sites. Median RT dose was 30.6 Gy (range, 21.6 to 48.6 Gy). Median follow-up duration was 62 months (range, 6 to 141 months). RESULTS: All patients showed complete response in the radiation field. Eight outfield relapses were reported. Seven patients received salvage treatment (three chemotherapy, four RT). Four patients showed excellent responses, especially to RT. Estimated 5-year and 10-year relapse-free survivals were 72% and 60%. In the RT-alone group, 5-year relapse-free survival was 74.5%. All advanced-stage patients were disease-free with 100% 5-year overall survival. Disease-specific death was noted in only one patient; four others died of other unrelated causes. No significant toxicity was reported. CONCLUSION: RT resulted in excellent treatment outcomes for all FL stages when used as a primary treatment modality for early stage or salvage-treatment modality for advanced-stage disease.
Drug Therapy ; Follow-Up Studies ; Humans ; Lymphoma, Follicular* ; Lymphoma, Non-Hodgkin ; Radiotherapy* ; Recurrence ; Retrospective Studies ; Treatment Outcome