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1.
The Korean Journal of Internal Medicine ; : 841-850, 2022.
Article in English | WPRIM | ID: wpr-939090

ABSTRACT

Background/Aims@#We evaluated the feasibility and long-term efficacy of the combination of cytarabine, idarubicin, and all-trans retinoic acid (ATRA) for treating patients with newly diagnosed acute promyelocytic leukemia (APL). @*Methods@#We included 87 patients with newly diagnosed acute myeloid leukemia and a t(15;17) or promyelocytic leukemia/retinoic acid receptor alpha (PML-RARα) mutation. Patients received 12 mg/m2/day idarubicin intravenously for 3 days and 100 mg/m2/day cytarabine for 7 days, plus 45 mg/m2/day ATRA. Clinical outcomes included complete remission (CR), relapse-free survival (RFS), overall survival (OS), and the secondary malignancy incidence during a 20-year follow-up. @*Results@#The CR, 10-year RFS, and 10-year OS rates were 89.7%, 94.1%, and 73.8%, respectively, for all patients. The 10-year OS rate was 100% for patients that achieved CR. Subjects were classified according to the white blood cell (WBC) count in peripheral blood at diagnosis (low-risk, WBC < 10,000/mm3; high-risk, WBC ≥ 10,000/mm3). The low-risk group had significantly higher RFS and OS rates than the high-risk group, but the outcomes were not superior to the current standard treatment (arsenic trioxide plus ATRA). Toxicities were similar to those observed with anthracycline plus ATRA, and higher than those observed with arsenic trioxide plus ATRA. The secondary malignancy incidence after APL treatment was 2.7%, among the 75 patients that achieved CR, and 5.0% among the 40 patients that survived more than 5 years after the APL diagnosis. @*Conclusions@#Adding cytarabine to anthracycline plus ATRA was not inferior to anthracycline plus ATRA alone, but it was not comparable to arsenic trioxide plus ATRA. The probability of secondary malignancy was low.

2.
Yonsei Medical Journal ; : 935-943, 2015.
Article in English | WPRIM | ID: wpr-40871

ABSTRACT

PURPOSE: Beroctocog alfa is a second generation recombinant factor VIII manufactured by removing the B-domain from factor VIII. This prospective clinical trial was conducted to evaluate the efficacy, safety, and pharmacokinetics of beroctocog alfa in patients of ages > or =12 years previously treated for severe hemophilia A. MATERIALS AND METHODS: Seventy subjects received beroctocog alfa as an on-demand treatment for acute hemorrhage. RESULTS: The final hemostatic effect was excellent in 35 subjects (50%) and good in 26 subjects (37.1%). The drug showed an overall efficacy rate of 87.1%. The majority of acute hemorrhages was treated by administering the study drug once (86.2%) or twice (10.0%), and the mean dose administered per single infusion was 28.55+/-6.53 IU/kg. Ten subjects underwent 12 surgical procedures, and hemostatic efficacy was excellent in seven cases (58.3%) and good in five cases (41.7%), showing a 100% efficacy rate. A total of 52 of 88 subjects (59.0%) experienced 168 adverse events. There were 18 serious adverse events (10.7%) in 11 subjects, and two (mild dyspnea and facial edema) in one subject were related to the study drug. Only one subject formed a de novo factor VIII inhibitor, for an occurrence rate of 1.4% (one-sided 95% upper confidence limit: 3.85%). The final elimination half-life was 13.3 h and 12.6 h at baseline and 6 months after administration, respectively. CONCLUSION: Our results suggest that beroctocog alfa is safe and efficacious as either an on-demand treatment for acute hemorrhage or a surgical prophylaxis in patients with hemophilia A.


Subject(s)
Adult , Female , Humans , Male , Middle Aged , Consumer Product Safety , Dyspnea , Factor VIII/adverse effects , Hemophilia A/drug therapy , Hemorrhage/prevention & control , Hemostasis , Hemostasis, Surgical/methods , Prospective Studies , Recombinant Proteins/adverse effects , Treatment Outcome
3.
Laboratory Medicine Online ; : 183-188, 2013.
Article in Korean | WPRIM | ID: wpr-228111

ABSTRACT

We present a case of abrupt-onset hemorrhagic tendency in a patient with amyloidosis who also had asymptomatic plasma cell myeloma. The patient was a 66-yr-old man with no previous history of hemorrhagic tendency and no family history of hemorrhagic disease. On examination, the prothrombin time and activated partial thromboplastin time were found to be prolonged and were not corrected even after a mixing test; moreover, the levels of coagulation factors I, II, V, VII, and X were almost normal. We therefore considered the presence of a nonspecific coagulation inhibitor. Although the von Willebrand factor (vWF) activity and vWF antigen level were normal due to sampling following transfusion, the increased closure time on PFA-100 (Siemens) analysis and the absence of ristocetin-induced platelet aggregation suggested the presence of acquired von Willebrand syndrome (vWS). After chemotherapy, the patient showed alleviation in the bleeding symptoms. Therefore, testing for acquired vWS should be considered when a patient has a history of recent bleeding with underlying amyloidosis.


Subject(s)
Humans , Amyloidosis , Blood Coagulation Factors , Hemorrhage , Light , Multiple Myeloma , Partial Thromboplastin Time , Plasma , Plasma Cells , Platelet Aggregation , Prothrombin Time , von Willebrand Factor
4.
Journal of Korean Medical Science ; : 851-858, 2011.
Article in English | WPRIM | ID: wpr-205261

ABSTRACT

Mesenchymal stem cells (MSCs) have the capacity to proliferate and differentiate into multiple connective tissue lineages, which include cartilage, bone, and fat. Cartilage differentiation and chondrocyte maturation are required for normal skeletal development, but the intracellular pathways regulating this process remain largely unclear. This study was designed to identify novel genes that might help clarify the molecular mechanisms of chondrogenesis. Chondrogenesis was induced by culturing human bone marrow (BM) derived MSCs in micromass pellets in the presence of defined medium for 3, 7, 14 or 21 days. Several genes regulated during chondrogenesis were then identified by reverse transcriptase-polymerase chain reaction (RT-PCR). Using an ABI microarray system, we determined the differential gene expression profiles of differentiated chondrocytes and BM-MSCs. Normalization of this data resulted in the identification of 1,486 differentially expressed genes. To verify gene expression profiles determined by microarray analysis, the expression levels of 10 genes with high fold changes were confirmed by RT-PCR. Gene expression patterns of 9 genes (Hrad6B, annexinA2, BMP-7, contactin-1, peroxiredoxin-1, heat shock transcription factor-2, synaptotagmin IV, serotonin receptor-7, Axl) in RT-PCR were similar to the microarray gene expression patterns. These findings provide novel information concerning genes involved in the chondrogenesis of human BM-MSCs.


Subject(s)
Humans , Bone Marrow Cells/cytology , Cell Differentiation , Chondrocytes/metabolism , Chondrogenesis/genetics , Gene Expression Profiling , Mesenchymal Stem Cells/cytology , Oligonucleotide Array Sequence Analysis , Reverse Transcriptase Polymerase Chain Reaction , Time Factors
5.
Korean Journal of Hematology ; : 310-314, 2009.
Article in Korean | WPRIM | ID: wpr-720066

ABSTRACT

Acquired von Willebrand syndrome (AvWS) is a relatively rare acquired bleeding disorder similar to inherited von Willebrand disease in terms of laboratory findings, and occurs without a personal or family history of bleeding. A 23-year-old man with no previous disease history and no family history of hemorrhagic diathesis was referred to our hospital because of recurrent epistaxis and intramuscular hematoma. He was diagnosed as having AvWS because of an almost complete absence of ristocetin cofactor activity (vWF : RCo) despite normal vWF antigen level. Furthermore, anti-vWF antibody was detected in his serum using home-brewed ELISA. Finally, the amyloid deposit was found in muscle biopsy. He was diagnosed with AvWS which is associated with amyloidosis. AvWS should be considered in patients with current bleeding diatheses and no past history of bleeding.


Subject(s)
Humans , Young Adult , Amyloidosis , Autoantibodies , Biopsy , Disease Susceptibility , Enzyme-Linked Immunosorbent Assay , Epistaxis , Hematoma , Hemorrhage , Hemorrhagic Disorders , Muscles , Plaque, Amyloid , von Willebrand Diseases , von Willebrand Factor
6.
Experimental & Molecular Medicine ; : 678-685, 2009.
Article in English | WPRIM | ID: wpr-76424

ABSTRACT

In spite of the importance of phospholipase D (PLD) in cell proliferation and tumorigenesis, little is known about the molecules regulating PLD expression. Thus, identification of small molecules inhibiting PLD expression would be an important advance for PLD-mediated physiology. We examined one such here, denoted "Triptolide", which was identified in a chemical screen for inhibitors of PLD expression using cell assay system based on measurement of PLD promoter activity. Triptolide significantly suppressed the expression of both PLD1 and PLD2 with sub-microM potency in MDA-MB-231 breast cancer cells as analyzed by promoter assay and RT-PCR. Moreover, triptolide abolished the protein level of PLD in a time and dose-dependent manner. Triptolide-induced PLD1 downregulation was also observed in all the cancer cells examined, suggesting a general phenomenon detected in various cancer cells. Decrease of PLD expression by triptolide suppressed both basal and PMA-induced PLD activity. In addition, triptolide inhibited activation of NFkappaB which increased PLD1 expression. Ultimately, downregulation of PLD by triptolide inhibited proliferation of breast cancer cells. Taken together, we demonstrate that triptolide suppresses the expression of PLD via inhibition of NFkappaB activation and then decreases cell proliferation.


Subject(s)
Female , Humans , Antineoplastic Agents, Alkylating/pharmacology , Breast Neoplasms/drug therapy , Cell Line, Tumor , Cell Proliferation/drug effects , Diterpenes/pharmacology , Epoxy Compounds/pharmacology , Gene Expression Regulation, Neoplastic/drug effects , NF-kappa B/genetics , Phenanthrenes/pharmacology , Phospholipase D/genetics
7.
Korean Journal of Hematology ; : 197-205, 2007.
Article in Korean | WPRIM | ID: wpr-720794

ABSTRACT

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.


Subject(s)
Adult , Female , Humans , Male , alpha-Thalassemia , Anemia, Hemolytic, Congenital , beta-Thalassemia , Bilirubin , Cell Membrane , Diagnosis , Elliptocytosis, Hereditary , Erythrocyte Indices , Hemoglobinopathies , Internal Medicine , Jaundice , Korea , L-Lactate Dehydrogenase , Oxidoreductases , Pallor , Pathology, Molecular , Pediatrics , Phosphopyruvate Hydratase , Prevalence , Pyruvate Kinase , Reticulocyte Count , Retrospective Studies , Sex Ratio , Surveys and Questionnaires
8.
Korean Journal of Hematology ; : 75-81, 2005.
Article in Korean | WPRIM | ID: wpr-720444

ABSTRACT

BACKGROUND: Curcumin, a naturally occurring biologically active compound extracted from rhizomes of Curcuma species, has been shown to possess potent anti-inflammatory, anti-tumor and anti-oxidative properties. The effects and possible mechanism of this agent were investigated on 2 human myelogenous leukemic cell lines. METHODS: K562 and KG-1 cells were the two cell lines selected. The MTT assay and flow cytometry were used to assess the cytotoxicity and for cell cycle analysis, respectively. The protein expressions were analyzed by Western blotting; the caspase activity was also checked. RESULTS: Both cell lines showed dose-dependent susceptibility to curcumin, and the cell cycle analysis showed an increased sub-G1 phase in the KG-1 cells. In the K562 cell, curcumin down regulated the expressions of PCNA (proliferating cell nuclear antigen) and cyclins D1 and B1. The expression of Akt was also down-regulated, but caspase-3 was activated to induce cleaved PARP (polyadenosine ribose polymerase) and apoptosis. However, the expression of phospho-Erk was unaffected. Co-treatment of cyclosporin A (CsA) with curcumin resulted in an attenuation of apoptosis in the K562 cells, implying curcumin-induced apoptosis is dependent on the release of cytochrome c from the mitochondria. CONCLUSION: Curcumin induced cell cycle arrest and apoptosis in both human myelogenous leukemic cell lines, with the apoptosis appearing to be dependent on the release of cytochrome c from the mitochondria.


Subject(s)
Humans , Apoptosis , Blotting, Western , Caspase 3 , Cell Cycle , Cell Cycle Checkpoints , Cell Line , Curcuma , Curcumin , Cyclins , Cyclosporine , Cytochromes c , Flow Cytometry , K562 Cells , Leukemia, Myeloid , Mitochondria , Proliferating Cell Nuclear Antigen , Rhizome , Ribose
9.
Korean Journal of Medicine ; : 437-441, 2004.
Article in Korean | WPRIM | ID: wpr-99258

ABSTRACT

During the last decade the issue of therapy-related myelodysplasia (t-MDS) and acute myeloid leukemia (t-AML) following high-dose chemotherapy (HD-CT) and autologous stem cell transplantation (ASCT) or conventional chemotherapy, radiotherapy for malignant diseases has become increasingly important. The number of patients with hematologic malignancies and chemosensitive or radiosensitive solid tumors undergoing this new type of treatment has expanded dramatically. Recently, we experienced a case of 35-year-old female patient with therapy-related acute lymphoblastic leukemia (t-ALL) after 131I treatment for thyroid papillary carcinoma. Total 131I dose the patient received was 900mCi and patient was diagnosed as having ALL 11 months after last 131I treatment. Although the prognosis of therapy-related acute leukemia/myelodysplastic syndrome is poor, the patient is alive in complete remission after allogeneic bone marrow transplantation from HLA-matched sibling donor. Given the rarity of this case, we report the case with literature reviews.


Subject(s)
Adult , Female , Humans , Bone Marrow Transplantation , Carcinoma, Papillary , Drug Therapy , Hematologic Neoplasms , Leukemia, Myeloid, Acute , Neoplasms, Second Primary , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Prognosis , Radiotherapy , Siblings , Stem Cell Transplantation , Thyroid Gland , Thyroid Neoplasms , Tissue Donors
10.
The Korean Journal of Internal Medicine ; : 21-28, 2003.
Article in English | WPRIM | ID: wpr-112362

ABSTRACT

BACKGROUND: Because of the relative paucity of data regarding the clinical outcome in adult patients with acute lymphocytic leukemia (ALL) in Korea, we analyzed clinical courses in adult ALL patients treated with VPD (L) regimen (vincristine, prednisolone, daunorubicin, L-asparaginase) at the Seoul National University Hospital, and evaluated prognostic factors influencing the outcome. METHODS: Patients with ALL newly diagnosed between October 1994 and June 2000 at our hospital were analyzed retrospectively. Fifty-three patients were evaluable. Induction chemotherapy consisted of VPD with (46 cases) or without L-asparaginase (7 cases). After complete remission (CR), consolidation therapy, CNS prophylaxis and maintenance chemotherapy were administered. RESULTS: Ages ranged from 16 to 67 (median 30). CR rate was 86.8% (46/53) and no significant prognostic factor was found for the CR rate. With a median follow-up time of 27.2 months (range 12.9~83.0 months) in living patients, the median overall survival (OS) for all cases was 16.7 months (13.4~20.1 months, 95% C.I.) and the estimated 4-year OS rate was 25.4%+/-8.9%. The median relapse-free survival (RFS) was 12.2 months (8.4~16.0 months, 95% C.I.), and 3-year RFS rate was 29.9%+/-10.2%. Poor prognostic factors for OS were Ph chromosome (p=0.005) and T-cell immunophenotype (p=0.03). For RFS they were Ph chromosome (p=0.01) and the presence of a mediastinal mass (p=0.03). CONCLUSION: Despite an initial excellent response to the VPD (L) regimen, newer therapeutic strategies, including more intensive postremission therapies, are urgently needed because of the high relapse rate. Future therapeutic approaches need to be stratified according to several prognostic factors.


Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Administration, Oral , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Asparaginase/administration & dosage , Disease-Free Survival , Dose-Response Relationship, Drug , Doxorubicin/administration & dosage , Drug Administration Schedule , Follow-Up Studies , Infusions, Intravenous , Maximum Tolerated Dose , Multivariate Analysis , Precursor Cell Lymphoblastic Leukemia-Lymphoma/diagnosis , Prednisone/administration & dosage , Probability , Remission Induction , Retrospective Studies , Survival Analysis , Treatment Outcome , Vincristine/administration & dosage
11.
Korean Journal of Hematology ; : 191-199, 2002.
Article in Korean | WPRIM | ID: wpr-720836

ABSTRACT

BACKGROUND: Flow cytometric measurement of DNA can reveal G0/G1, S, G2/M phases of cell cycle, and BrdU labeling can determine the percentage of cells in active DNA synthesis. A monoclonal antibody (MoAb), Ki-67, recognizes a protein that is present only in the nucleus of cycling cells but absent in resting cells. We analyzed whether the resting and the proliferating fraction could be differentiated by double staining with Ki-67 MoAb and propidium iodide (PI), and observed the effects of GM-CSF on cell cycle in acute myelogenous leukemia (AML) cells by Ki-67 MoAb. METHODS: Blast cells were prepared from 9 AML patients. The cells were incubated for 48 hours with or without GM-CSF. Cells were stained with BrdU/PI and Ki-67/PI. Cell cycle was analyzed by flow cytometry. RESULTS: The average fraction of G0/G1, S, and G2/M phases was 84.6%, 10.9%, and 4.5 % by BrdU/PI and 87.8%, 8.6%, and 3.7% by Ki-67/PI, respectively. Ki-67/PI staining dis-criminated between G0 and G1 phases and the average was 71.5% and 16.3%, respectively. In cells incubated with GM-CSF, BrdU/ PI method showed that the average S phase fraction (SPF) significantly increased from 10.9 to 16.2% (P=0.01) and the fraction of G0/G1 phase decreased from 84.6% to 78.4% (P= .02). Ki-67/PI method showed that the median SPF significantly increased from 8.6% to 13.7% (P=0.05) and G0 fraction decreased from 71.5% to 58.1% (P=0.02) but G1 fraction increased from 16.3% to 22.3% (P=0.01). CONCLUSION: Cell cycle analysis by Ki-67 MoAb and PI in AML is rapid and simple. It is especially useful to determine the growth fraction and G0 fraction compared to BrdU/PI staining.


Subject(s)
Humans , Bromodeoxyuridine , Cell Cycle , DNA , Flow Cytometry , Resting Phase, Cell Cycle , G1 Phase , Granulocyte-Macrophage Colony-Stimulating Factor , Ki-67 Antigen , Leukemia, Myeloid, Acute , Propidium , S Phase
12.
Korean Journal of Hematology ; : 259-264, 2002.
Article in English | WPRIM | ID: wpr-720828

ABSTRACT

BACKGROUND: There are few therapeutic options for patients with multiple myeloma who relapse after autologous or allogeneic stem cell transplantation, or for patients who are refractory to conventional chemotherapy and not eligible for salvage high-dose therapy. Thalidomide, a potent antiangiogenic agent, has been suggested as an effective salvage therapy in refractory multiple myeloma. The aim of this study was to evaluate the efficacy and tolerance of thalidomide as a single agent as multicenter trial in Korea. METHOD: From February 2001 to September 2002, 28 patients from 4 institutions were included. At start of treatment, all patients had active disease and 17 (61%) had received at least one autologous transplantation. RESULTS: The serum or urine levels of paraprotein were reduced by at least 90 percent in two patients, at least 50 percent in three patients, and at least 25 percent in two patients; for a total response rate of 25 percent. 13 patients had stable disease and 8 patients had progressed. At least half of the patients had mild or moderate constipation and fatigue. More severe adverse effects were infrequent. CONCLUSION: This study confirms that thalidomide is an effective and safe agent in patients with relapsed or refractory multiple myeloma.


Subject(s)
Humans , Autografts , Constipation , Drug Therapy , Fatigue , Korea , Multiple Myeloma , Recurrence , Salvage Therapy , Stem Cell Transplantation , Thalidomide , Transplantation, Autologous
13.
Korean Journal of Nephrology ; : 443-449, 2002.
Article in Korean | WPRIM | ID: wpr-162512

ABSTRACT

BACKGROUND: Common complications after hematopoietic stem cell transplantation(HCT) include sepsis, graft versus host disease(GVHD), veno-occlusive disease(VOD), drug-induced nephrotoxicity, and acute renal failure(ARF). Prior studies report that the presence of ARF affects prognosis. However, we are unaware of such reports on the incidence of ARF after HCT in Koreans, and whether or not the development of ARF is related to prognosis. The purpose of our study was to investigate the cause of ARF after HCT and its relation to prognosis. METHODS: 163 patients received HCT at Seoul National University Hospital since 1985, of which, 107 were available for review. RESULTS: ARF after HCT developed in 52 patients (48.6%). In the three clinical causes, VOD, sepsis, and GVHD, risk factor related to the development of ARF was preexisting VOD. Logistic regression confirmed this association(odds ratio 4.4). The causes of ARF were different according to the periods it developed, and cyclosporin nephrotoxicity was the main cause through the whole period after HCT. The overall survival was worse in the ARF group(60 vs 73 %; p < 0.05). ARF group was split into two groups : patients whose peak serum creatinine levels were below 3.0 mg/dL(mild ARF group) and those who were above 3.0 mg/dL(severe ARF group). Severe ARF group had worse survival than mild ARF group and patients without ARF(p < 0.01). CONCLUSION: VOD, sepsis, GVHD after HCT increase the risk of the deveolopment of ARF, but cyclosprin nephrotoxicity is the main cause of ARF. Severe ARF is a factor influencing the prognosis of patients who received HCT.


Subject(s)
Humans , Acute Kidney Injury , Cell Transplantation , Creatinine , Cyclosporine , Hematopoietic Stem Cell Transplantation , Hematopoietic Stem Cells , Incidence , Logistic Models , Prognosis , Renal Insufficiency , Risk Factors , Seoul , Sepsis , Transplants
14.
The Korean Journal of Laboratory Medicine ; : 373-381, 2002.
Article in Korean | WPRIM | ID: wpr-55460

ABSTRACT

BACKGROUND: The complementary ABL-BCR gene rearrangement is formed at chromosome 9 parallel to the Ph chromosome at der(22)t(9;22), which has been found deleted in a minority of chronic myelogenous leukemia (CML) patients. This study was designed to analyze the deletion status of ABL and/or BCR on derivative chromosome 9 and to evaluate the prognostic significance of the deletion of these genes in CML. METHODS: We studied 79 patients who were diagnosed as CML at Seoul National University Hospital between January 1997 and February 2002. The deletion status of ABL and BCR on derivative chromosome 9 was investigated by interphase fluorescent in situ hybridization (FISH) method. RESULTS: ABL deletion was detected in 14 (17.7%) patients and BCR deletion was observed in 8 patients (10.1%). Event-free survival time of the patients with ABL and/or BCR deletion (19.0%) was shorter than that of the patients without any deletion of these two genes (median, 40.0 months vs. 92.0 months)(P=0.027). Twenty seven patients progressed to blast crisis in this period. The period to blast crisis was also shorter in 8 patients with ABL and/or BCR deletion than in 19 patients without any gene deletion (P=0.044). The b2a2 mRNA type was more frequent in the patients with ABL deletion only than in the patients without any gene deletion (P=0.034). CONCLUSIONS: Event-free survival time and the period to blast crisis were significantly shorter in patients with deletion of ABL and/or BCR on derivative chromosome 9. ABL and/or BCR deletion can be a significant prognostic marker that indicates rapid disease progression.


Subject(s)
Humans , Blast Crisis , Chromosomes, Human, Pair 9 , Disease Progression , Disease-Free Survival , Gene Deletion , Gene Rearrangement , Hydrogen-Ion Concentration , In Situ Hybridization, Fluorescence , Interphase , Leukemia, Myelogenous, Chronic, BCR-ABL Positive , Philadelphia Chromosome , RNA, Messenger , Seoul
15.
Korean Journal of Infectious Diseases ; : 331-336, 2002.
Article in Korean | WPRIM | ID: wpr-71903

ABSTRACT

Pneumonia, along with graft-versus-host disease, is a major cause of morbidity and mortality in patients receiving bone marrow transplantation. The community respiratory virus infections have been found to be large causes of pneumonia. Upper respiratory infection with Parainfluenza virus can progress to severe lower respiratory diseases in bone marrow transplant recipients, of which clinical findings are similar to those of pneumonia by exotic opportunistic pathogens. We report a patient with chronic myelogenous leukemia who had suffered a community-acquired pneumonia by Parainfluenza virus type 3 after bone marrow transplantation.


Subject(s)
Humans , Bone Marrow Transplantation , Bone Marrow , Graft vs Host Disease , Leukemia , Leukemia, Myelogenous, Chronic, BCR-ABL Positive , Mortality , Parainfluenza Virus 3, Human , Paramyxoviridae Infections , Pneumonia , Transplantation
16.
Korean Journal of Medicine ; : 241-242, 2002.
Article in Korean | WPRIM | ID: wpr-135767

ABSTRACT

No abstract available.

17.
Korean Journal of Medicine ; : 241-242, 2002.
Article in Korean | WPRIM | ID: wpr-135762

ABSTRACT

No abstract available.

18.
Korean Journal of Clinical Pathology ; : 315-322, 2001.
Article in Korean | WPRIM | ID: wpr-29246

ABSTRACT

BACKGROUND: Translocations involving the MLL gene on the long arm of chromosome 11 (11q23) are frequently observed in acute leukemia. The detection of this genetic change has a unique significance due to its implication for poor prognosis. The aim of this study was to determine the utility of fluorescence in situ hybridization (FISH) method in detecting the MLL translocation. METHODS: We applied both conventional cytogenetic analysis (CC) and MLL FISH on 289 consecutive Korean patients (children and adults) with acute leukemia and analyzed the data, placing an emphasis on the discrepancies in the results. RESULTS: Twenty-two of 289 patients (7.6%) had the 11q23/MLL translocation. In 9 cases of 22 (41%), only FISH detected the translocation. In 8 among 22 patients, a total of 19 follow-up examinations were performed, of which FISH detected a significant level of leukemia cells harboring the MLL translocation in 5 (26%) without cytogenetic evidence. Besides the MLL translocation, FISH detected submicroscopic amplification, partial deletion of the MLL gene, and trisomy 11 in 12 cases without cytogenetic evidence. CONCLUSIONS: These results demonstrate that up to 41% of the MLL translocations at initial workups and 26% during follow-up were detected by FISH without cytogenetic evidence. Thus, we recommend that MLL FISH should be performed in the diagnosis and monitoring of acute leukemia in combination with CC.


Subject(s)
Humans , Arm , Asian People , Chromosomes, Human, Pair 11 , Cytogenetic Analysis , Cytogenetics , Diagnosis , Fluorescence , Follow-Up Studies , In Situ Hybridization , Leukemia , Prognosis , Trisomy
19.
Journal of the Korean Radiological Society ; : 339-343, 2001.
Article in Korean | WPRIM | ID: wpr-16783

ABSTRACT

PURPOSE: To describe the clinical and radiologic findings of all-trans-retinoic acid (ATRA) syndrome in acute promyelocytic leukemia. MATERIALS AND METHODS: Among 21 patients with acute promyelocytic leukemia who were treated with all-trans- retinoic acid between 1995 and 1998, we retrospectively evaluated the cases of four with ATRA syn-drome. Two were male and two were female, and their mean age was 58 years. The clinical and radiologic findings of chest radiography (n=4) and HRCT (n=1) were analyzed. RESULTS: Between seven and 13 days after ATRA treatment, dry cough, dyspnea and high fever developed in all patients. The WBC count in peripheral blood was significantly higher [2.9 -25.3(mean, 10.8)-fold] than before ATRA treatment, and in all patients, chest radiography revealed ill-defined consolidation and pleural effu-sion. Kerley 's B line (n=3) and hilar enlargement (n=3) were also seen, and in one patient, HRCT demonstrated septal line thickening. Among four patients treated with prednisolone and Ara-C,three recovered and one CONCLUSION: In acute promyelocytic patients treated with all-trans-retinoic acid, radiologic findings of ill-de-fined consolidation, pleural effusion, hilar prominence and Kerley 's B line may suggest ATRA syndrome. The early diagnosis of this will improve the patients' prognosis.


Subject(s)
Female , Humans , Male , Cough , Dyspnea , Early Diagnosis , Fever , Leukemia, Promyelocytic, Acute , Pleural Effusion , Prednisolone , Prognosis , Radiography , Retrospective Studies , Thorax , Tretinoin
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