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1.
Clinical and Molecular Hepatology ; : 110-124, 2021.
Artigo em Inglês | WPRIM | ID: wpr-874444

RESUMO

Background/Aims@#Nonalcoholic fatty liver disease (NAFLD) is closely related to gut-microbiome. There is a paucity of research on which strains of gut microbiota affect the progression of NAFLD. This study explored the NAFLD-associated microbiome in humans and the role of Lactobacillus in the progression of NAFLD in mice. @*Methods@#The gut microbiome was analyzed via next-generation sequencing in healthy people (n=37) and NAFLD patients with elevated liver enzymes (n=57). Six-week-old male C57BL/6J mice were separated into six groups (n=10 per group; normal, Western, and four Western diet + strains [109 colony-forming units/g for 8 weeks; L. acidophilus, L. fermentum, L. paracasei, and L. plantarum]). Liver/body weight ratio, liver pathology, serum analysis, and metagenomics in the mice were examined. @*Results@#Compared to healthy subjects (1.6±4.3), NAFLD patients showed an elevated Firmicutes/Bacteroidetes ratio (25.0±29.0) and a reduced composition of Akkermansia and L. murinus (P<0.05). In the animal experiment, L. acidophilus group was associated with a significant reduction in liver/body weight ratio (5.5±0.4) compared to the Western group (6.2±0.6) (P<0.05). L. acidophilus (41.0±8.6), L. fermentum (44.3±12.6), and L. plantarum (39.0±7.6) groups showed decreased cholesterol levels compared to the Western group (85.7±8.6) (P<0.05). In comparison of steatosis, L. acidophilus (1.9±0.6), L. plantarum (2.4±0.7), and L. paracasei (2.0±0.9) groups showed significant improvement of steatosis compared to the Western group (2.6±0.5) (P<0.05). @*Conclusions@#Ingestion of Lactobacillus, such as L. acidophilus, L. fermentum, and L. plantarum, ameliorates the progression of nonalcoholic steatosis by lowering cholesterol. The use of Lactobacillus can be considered as a useful strategy for the treatment of NAFLD.

2.
The Journal of the Korean Orthopaedic Association ; : 552-557, 2018.
Artigo em Coreano | WPRIM | ID: wpr-718965

RESUMO

Vitamin D plays a major role in bone metabolism, and its deficiency has an impact on fracture risk and healing. Low vitamin D levels are a cause of poor bone mineralization and have been associated with a significantly higher risk of physeal injury in children. This paper presents a case of a 13-year-old boy with a vitamin D deficiency, who sustained multiple sequential epiphyseal injuries at various areas. This report suggests that vitamin D deficiency is not only a significant cause of the clinical disease itself, but also an important factor affecting the successful recovery of injuries.


Assuntos
Adolescente , Criança , Humanos , Masculino , Calcificação Fisiológica , Metabolismo , Deficiência de Vitamina D , Vitamina D , Vitaminas
3.
Korean Journal of Hematology ; : 253-257, 2011.
Artigo em Inglês | WPRIM | ID: wpr-720156

RESUMO

BACKGROUND: The aim of this study is to investigate the hematological manifestations of human immunodeficiency virus (HIV) infection, the risk factors for cytopenia, and the effect of highly active anti-retroviral therapy (HAART) on cytopenia. METHODS: Medical records of patients treated for HIV at the Seoul National University Hospital from January 2005 to March 2010 were retrospectively reviewed. To determine the impact of HIV itself, we excluded HIV patients who had other conditions that could have resulted in hematological manifestations. Multiple logistic regression analyses were performed to identify risk factors for cytopenia. RESULTS: A total of 621 cases were investigated, and after exclusion, data of 472 patients were analyzed. The frequency of cytopenia was anemia, 3.0% (14/472); neutropenia, 10.0% (47/472); thrombocytopenia, 2.4% (12/472); lymphopenia, 25.7% (121/470); isolated cytopenia, 11.2% (53/472); and bicytopenia, 2.1% (10/472). The leading risk factor for cytopenia identified by multivariate logistic regression methods was AIDS status at initial presentation. After HAART, cytopenia was reversed in the majority of patients (thrombocytopenia, 100%; neutropenia, 91.1%; and anemia, 84.6%). CONCLUSION: This study isolated the impact of HIV infection alone on hematologic manifestations and confirmed that these changes were reversible by HAART. Control of the HIV infection will have the main role in the management of hematological manifestations of the virus.


Assuntos
Humanos , Anemia , Terapia Antirretroviral de Alta Atividade , HIV , Infecções por HIV , Modelos Logísticos , Linfopenia , Prontuários Médicos , Neutropenia , Estudos Retrospectivos , Fatores de Risco , Trombocitopenia , Vírus
4.
The Korean Journal of Laboratory Medicine ; : 1-8, 2011.
Artigo em Inglês | WPRIM | ID: wpr-30870

RESUMO

BACKGROUND: Heparin-induced thrombocytopenia (HIT) is an adverse drug reaction caused by antibodies to the heparin/platelet factor 4 (PF4) complex, resulting in thrombocytopenia and prothrombotic state. HIT diagnosis is challenging and depends on clinical presentation and laboratory tests. We investigated the usefulness of clinical scores and heparin/PF4 ELISA optical density (OD) as a diagnostic marker and thrombosis predictor in HIT. METHODS: We analyzed 92 patients with suspected HIT. The heparin/PF4 antibody was measured using a commercial ELISA kit (GTI, USA). For each patient, the 4 T's score and Chong's score were calculated. RESULTS: Of the 92 patients, 28 were anti-heparin/PF4-seropositive. The 4 T's score and Chong's score showed good correlation (r=0.874). The 4 T's score and OD values showed good performance for diagnosis of the definite and unlikely HIT groups; however, OD levels showed better sensitivity (93.8%) than the 4 T's score used alone (62.5%). Of the 92 patients, 26 developed thrombosis. The OD values were significantly higher in patients with thrombosis than in those without thrombosis (0.52 vs. 0.22, P0.4) had an increased risk of thrombosis (adjusted odds ratio 9.44 [3.35-26.6], P<0.001) and a shorter 250-day thrombosis-free survival (32.1% vs. 54.7%, P=0.012). CONCLUSIONS: ELISA OD values in combination with clinical scoring can improve the diagnosis of and thrombosis prediction in HIT. More attention should be paid to the use of clinical scores and OD values as thrombosis predictors in HIT.


Assuntos
Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Anticorpos/efeitos adversos , Área Sob a Curva , Ensaio de Imunoadsorção Enzimática/métodos , Heparina/imunologia , Fator Plaquetário 4/imunologia , Risco , Sensibilidade e Especificidade , Análise de Sobrevida , Trombocitopenia/induzido quimicamente , Trombose/diagnóstico
5.
The Korean Journal of Internal Medicine ; : 328-339, 2011.
Artigo em Inglês | WPRIM | ID: wpr-35155

RESUMO

BACKGROUND/AIMS: We investigated the clinical characteristics and prognosis of elderly patients with acute lymphoblastic leukemia (ALL). METHODS: We reviewed the clinical data, laboratory findings, bone marrow findings, and cytogenetic analysis of elderly patients (> or = 60 years) with ALL, and data of an additional 101 younger adult patients ( or = 60 years) and 101 younger adult patients ( or = 60 years) was 10.3 months (p = 0.003). In the elderly patients with ALL, T cell lineage and the presence of lymphadenopathy were significant prognostic factors for OS in a univariate analysis (p = 0.033 and 0.041, respectively). CONCLUSIONS: The outcomes of Korean elderly patients with ALL were poor, and the shorter OS was mainly due to the low CR rate. T-cell lineage and the presence of lymphadenopathy were significant prognostic factors in Korean elderly patients with ALL.


Assuntos
Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Fatores Etários , Exame de Medula Óssea , Distribuição de Qui-Quadrado , Intervalo Livre de Doença , Estimativa de Kaplan-Meier , Modelos Logísticos , Cromossomo Filadélfia , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Modelos de Riscos Proporcionais , Indução de Remissão , República da Coreia , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Taxa de Sobrevida , Fatores de Tempo , Resultado do Tratamento
6.
Korean Journal of Hematology ; : 269-274, 2010.
Artigo em Inglês | WPRIM | ID: wpr-720654

RESUMO

BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder characterized by chronic complement-mediated hemolysis. Eculizumab, a humanized monoclonal antibody against the terminal complement protein C5, potently reduces chronic intravascular hemolysis. We tested the clinical efficacy and safety of a 24-week treatment with eculizumab in 6 Korean patients with PNH. METHODS: We enrolled 6 patients with PNH who had clinically significant hemolysis. Eculizumab was administered intravenously at 600 mg/week for the first 4 weeks followed by 900 mg at week 5 and 2nd weekly thereafter. RESULTS: Three men and 3 women with a median age of 39.5 years (24-61 years) were enrolled. The median duration of PNH was 11 years (6-25 years). Hemolysis occurred in all patients [median lactate dehydrogenase (LDH) level, 7.95 times the upper limit of the reference range of LDH]. All patients treated with eculizumab had a rapid and sustained reduction in the degree of hemolysis. RBC transfusion requirements for 3 months were decreased from 0-12 units (median requirement, 1.5 units) to 0-6 units (median requirement, 0 units). Improvement in fatigue was noted in 4 patients. Further, 5 patients who had been receiving corticosteroids either reduced the dose or discontinued therapy. No significant adverse events related to eculizumab therapy were observed. CONCLUSION: These results show that eculizumab reduces the degree of intravascular hemolysis, reduces or eliminates the requirement of RBC transfusion, and improves anemia and fatigue in patients with PNH. Eculizumab is an effective and safe option for treating Korean patients with PNH.


Assuntos
Feminino , Humanos , Masculino , Corticosteroides , Anemia , Anticorpos Monoclonais Humanizados , Proteínas do Sistema Complemento , Fadiga , Hemoglobinúria Paroxística , Hemólise , L-Lactato Desidrogenase , Valores de Referência
7.
Korean Journal of Hematology ; : 95-101, 2010.
Artigo em Inglês | WPRIM | ID: wpr-720274

RESUMO

BACKGROUND: The role of pre-transplant salvage chemotherapy has been controversial in relapsed acute leukemia. METHODS: We investigated post-transplant outcomes in 65 patients with acute leukemia treated with allogeneic hematopoietic cell transplantation (HCT) during first relapse or second remission. RESULTS: The 5-year cumulative incidence of relapse (CIR) was 52.3%. Multivariate analysis for CIR revealed that patients with unfavorable cytogenetics and those not in remission at the time of HCT had a significantly high CIR (P = 0.031 and P = 0.031, respectively). Allogeneic HCT was performed in 14 patients after first relapse without salvage chemotherapy ("untreated relapse" group), 15 patients failed chemotherapy for reinduction of remission before HCT ("refractory relapse" group), and 36 patients attained second remission with salvage chemotherapy before HCT ("second remission" group). The 5-year CIR for patients in the untreated relapse group (57.1%) was higher than that for those in the second remission group (42.3%), but it was lower than that for patients in the refractory relapse group (66.7%). Among patients who underwent allogeneic HCT in relapse, those with bone marrow (BM) blasts 30%) (57.7% vs. 70.6%). CONCLUSION: Our results do not support the role of salvage chemotherapy aimed at re-induction of remission before allogeneic HCT in patients with acute leukemia after first relapse. Patients with early relapse do not appear to benefit from salvage chemotherapy before HCT.


Assuntos
Humanos , Medula Óssea , Transplante de Células , Citogenética , Incidência , Leucemia , Análise Multivariada , Recidiva , Transplantes
8.
Journal of Korean Medical Science ; : 781-784, 2010.
Artigo em Inglês | WPRIM | ID: wpr-157568

RESUMO

A 16-yr-old girl received liver transplantation for fulminant hepatitis. Aplastic anemia developed, and she received hematopoietic stem cell transplantation (HSCT). Eleven months after liver transplantation, abdominal lymph node enlargement and colon ulcers were observed, and colon biopsy showed posttransplant lymphoproliferative disorder (PTLD). Immunosuppression reduction was attempted, but it produced no therapeutic effect. Fourteen months after liver transplantation, she received a second HSCT due to engraftment failure, and PTLD resolved completely. The second HSCT can serve as cellular therapy for PTLD.


Assuntos
Adolescente , Feminino , Humanos , Transplante de Células-Tronco Hematopoéticas/métodos , Transplante de Fígado/efeitos adversos , Transtornos Linfoproliferativos/etiologia , Resultado do Tratamento
9.
Korean Journal of Hematology ; : 320-324, 2009.
Artigo em Inglês | WPRIM | ID: wpr-721041

RESUMO

Systemic amyloidosis is a disease that displays deposition of insoluble polymeric protein fibrils in tissues and organs. We report here on a case of a 64-year-old woman who initially presented with multiple enlarged lymph nodes. Computed tomography showed multiple enlarged lymph nodes in the mediastinal, lower cervical, supraclavicular, axillary and abdominal areas. Excision biopsy of the cervical lymph nodes and the subsequent histopathology showed amorphous eosinophilic material deposits, and these revealed apple-green birefringence on a polarizing microscopic examination on the Congo-red stained slide. The patient was diagnosed with amyloidosis and she received chemotherapy consisting of melphalan and dexamethasone. During chemotherapy, she was diagnosed with breast cancer. After modified unilateral radical mastectomy, the dexamethasone was restarted and this therapy resulted in stable disease.


Assuntos
Feminino , Humanos , Pessoa de Meia-Idade , Amiloide , Amiloidose , Biópsia , Birrefringência , Neoplasias da Mama , Dexametasona , Eosinófilos , Linfonodos , Doenças Linfáticas , Mastectomia Radical , Melfalan , Polímeros
10.
Korean Journal of Hematology ; : 100-103, 2009.
Artigo em Coreano | WPRIM | ID: wpr-720044

RESUMO

The blast phase in chronic myelogenous leukemia (CML) is associated with mutation of several genes. It is well known that p53 gene mutation plays a key role in the myeloid or lymphoid blast phase of CML. But for the case of the N-ras gene, the association between N-ras mutations and the blast phase of CML is not yet known. We report here on a case of detecting N-ras point mutation without p53 mutation in a 64 year-old man who suffered from the lymphoblastic blast phase of CML.


Assuntos
Humanos , Crise Blástica , Genes p53 , Genes ras , Leucemia Mielogênica Crônica BCR-ABL Positiva , Mutação Puntual , Estresse Psicológico
11.
The Korean Journal of Internal Medicine ; : 33-36, 2009.
Artigo em Inglês | WPRIM | ID: wpr-110932

RESUMO

BACKGROUND/AIMS: The financial burden of caring for iron-related complications (IRCs) is an emerging medical problem in Korea, as in Western countries. We produced a preliminary estimate of the costs of treating patients for IRCs. METHODS: The medical records of patients who had received multiple transfusions were reviewed. Newly developed cardiomyopathy, heart failure, diabetes mellitus, liver cirrhosis, and liver cancer were defined as IRCs. The costs of laboratory studies, medication, oxygenation, intervention, and education were calculated using working criteria we defined. Costs that had a definite causal relationship with IRCs were included to produce as accurate an estimate as possible. RESULTS: Between 2002 and 2006, 650 patients with hematologic diseases, including 358 with acute leukemia, 102 with lymphoma, 58 with myelodysplastic syndrome or myeloproliferative disease, 46 with multiple myeloma, and 31 with chronic leukemia, received more than 10 units of red blood cells. Nine patients developed IRCs. The primary diagnoses of eight patients were aplastic anemia and that of one patient was chronic lymphocytic leukemia. Two patients who had diabetes were excluded because one was treated at another hospital and the other was diagnosed as oxymetholone-induced diabetes. Of the seven patients included, liver cirrhosis developed in two, heart failure in four, and diabetes mellitus in three. Some of them had two diagnoses. The total cost attributed to IRCs for the seven patients was 47,388,241 KRW (approximately 50,000 USD). CONCLUSIONS: The medical costs of IRCs are considerable, and more effective iron-chelating therapy is necessary to save medical resources and improve patient care. More in the way of comprehensive health and economic studies of IRCs are needed to allow both clinicians and health-policy makers to make better decisions.


Assuntos
Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Custos e Análise de Custo/métodos , Transfusão de Eritrócitos/efeitos adversos , Custos de Cuidados de Saúde/estatística & dados numéricos , Doenças Hematológicas/terapia , Ferro/sangue , Quelantes de Ferro/economia , Sobrecarga de Ferro/economia , Coreia (Geográfico) , Estudos Retrospectivos
12.
Korean Journal of Hematology ; : 19-27, 2008.
Artigo em Coreano | WPRIM | ID: wpr-720817

RESUMO

BACKGROUND: Curcumin is a naturally occurring biologically active compound, and it has been shown to possess potent anti-inflammatory, anti-tumor and anti-oxidative properties. It is known for its anti-proliferative and proapoptotic effects in several cancer cells. Curcumin's effects on the mechanisms of cell survival and the expression of various cytokines were investigated in U266 cells and the in vivo effects of curcumin were examined using an animal model. METHODS: Cell proliferation assay and flow cytometry were used to examine cell proliferation, along with cell cycle analysis. The protein expressions were analyzed by Western blotting and the expressed levels of cytokines were analyzed by the ELISA method. RESULTS: Curcumin inhibited U266 cell growth in a dose-dependent and time-dependent manner. Cell cycle analysis showed an increased sub-G1 phase, a down regulated cyclinD1 expression and an induced p21 expression. Apoptosis induced a down regulated procaspase 3 expression and it induced cleaved PARP. Curcumin inhibited the IL (interleukin)-6 induced cell signal pathway via decreasing the STAT1 an 3, Erk cyclinD1 and c-myc expressions. Also, administration of 25mg/kg curcumin to a U266 animal model inhibited cancer cell engraftment in the bone marrow and it decreased the IL-6, sIL-6R and IL-8 expression levels. CONCLUSION: Curcumin induced cell cycle arrest and apoptosis and it inhibited the IL-6 mediated signal transduction pathways in U266 cells. Similar to the in vitro results, curcumin inhibited cancer cell proliferation and the expression of cytokine in vivo.


Assuntos
Animais , Apoptose , Western Blotting , Medula Óssea , Caspase 3 , Ciclo Celular , Pontos de Checagem do Ciclo Celular , Proliferação de Células , Sobrevivência Celular , Curcumina , Citocinas , Ensaio de Imunoadsorção Enzimática , Citometria de Fluxo , Interleucina-6 , Interleucina-8 , Modelos Animais , Mieloma Múltiplo , NF-kappa B , Peptídeos , Transdução de Sinais
13.
Journal of Korean Medical Science ; : 324-327, 2008.
Artigo em Inglês | WPRIM | ID: wpr-173545

RESUMO

We describe a 37-yr-old man who developed central pontine myelinolysis (CPM) after allogeneic hematopoietic stem cell transplantation (HSCT) for acute lymphoblastic leukemia. After HSCT, desquamation developed on the whole body accompanied by hyperbilirubinemia. The liver biopsy of the patient indicated graft-versus-host disease- related liver disease, and the dose of methylprednisolone was increased. Then, the patient developed altered mentality with eye ball deviation to the left, for which electroencephalogram and magnetic resonance imaging (MRI) scans were done. Brain MRI scan demonstrated the imaging findings consistent with central pontine myelinolysis and extrapontine myelinolysis. He did not have any hyponatremia episode during hospitalization prior to the MRI scan. To the best of our knowledge, presentation of CPM after allogeneic HSCT is extremely rare in cases where patients have not exhibited any episodes of significant hyponatremia. We report a rare case in which hepatic dysfunction due to graft-versus-host disease has a strong association with CPM after HSCT.


Assuntos
Adulto , Humanos , Masculino , Biópsia , Encéfalo/patologia , Eletroencefalografia , Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Hiperbilirrubinemia/etiologia , Fígado/patologia , Imageamento por Ressonância Magnética , Mielinólise Central da Ponte/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Fatores de Tempo , Resultado do Tratamento
14.
The Korean Journal of Internal Medicine ; : 40-44, 2007.
Artigo em Inglês | WPRIM | ID: wpr-199145

RESUMO

Pneumatosis intestinalis (PI) is an uncommon disorder characterized by an accumulation of gas in the bowel wall, and has been associated with a variety of disorders and procedures. We describe a 35-year-old man who undertook hematopoietic stem cell transplantation due to myelodysplastic syndrome. An abdominal X-ray demonstrated extensive PI with pneumoperitoneum mimicking hollow organ perforation. However, the patient had no abdominal symptoms and there was no evidence of peritoneal inflammation. After two weeks of conservative management, including bowel rest and antibiotics, his pneumoperitoneum resolved spontaneously without any complications. Of the many factors that affect the gastrointestinal tract mucosal integrity, intramural pressure, and bacterial flora-produced intraluminal gas interact to produce PI. If the condition is accompanied by bowel ischemia, portomesenteric venous gas, metabolic acidosis, and abdominal sepsis, or if PI is severe in extent immediate surgical intervention is indicated. The described case supports that a mechanical rather than a bacterial etiology underlies the pathogenesis of PI.


Assuntos
Masculino , Humanos , Adulto , Pneumoperitônio/diagnóstico , Pneumatose Cistoide Intestinal/diagnóstico , Síndromes Mielodisplásicas/diagnóstico , Perfuração Intestinal/diagnóstico , Transplante de Células-Tronco Hematopoéticas , Diagnóstico Diferencial
15.
Korean Journal of Hematology ; : 197-205, 2007.
Artigo em Coreano | WPRIM | ID: wpr-720794

RESUMO

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.


Assuntos
Adulto , Feminino , Humanos , Masculino , Talassemia alfa , Anemia Hemolítica Congênita , Talassemia beta , Bilirrubina , Membrana Celular , Diagnóstico , Eliptocitose Hereditária , Índices de Eritrócitos , Hemoglobinopatias , Medicina Interna , Icterícia , Coreia (Geográfico) , L-Lactato Desidrogenase , Oxirredutases , Palidez , Patologia Molecular , Pediatria , Fosfopiruvato Hidratase , Prevalência , Piruvato Quinase , Contagem de Reticulócitos , Estudos Retrospectivos , Razão de Masculinidade , Inquéritos e Questionários
16.
Korean Journal of Hematology ; : 233-240, 2007.
Artigo em Coreano | WPRIM | ID: wpr-720790

RESUMO

BACKGROUND: Donor lymphocyte infusion (DLI) has been established as a salvage therapy for patients with relapsed leukemia after allogeneic hematopoietic stem cell transplantation (HSCT). However, its benefit can be limited by the development of graft-versus-host disease (GVHD) or marrow aplasia. METHODS: We retrospectively analyzed the data from 39 patients that received DLI for relapsed leukemia after HLA-matched, related HSCT between 1995 and 2005 at Seoul National University Hospital. RESULTS: The diagnoses were CML (n=8), AML (n=19) and ALL (n=12). Ten patients had received non- myeloablative HSCT (AML=9, ALL=1). Complete remission after DLI was achieved in 6 (75%) cases with CML, 5 cases (29%) with AML and 5 cases (41%) with ALL. The two-year progression-free survival was 60% in CML patients, but 8.1% in non-CML patients (P=0.01). In addition, better overall survival (OS) was shown in CML patients than in non-CML patients (2-year OS, 68% in CML; 10% in non-CML, P=0.01). The durable remission for more than three years after DLI was confirmed in five patients (one AML patient for 88 months, one ALL patient for 54 months, three CML patients for 38, 47 and 53 months). Acute GVHD (> or =Grade II) developed in 14 patients (35.9%). Prolonged marrow aplasia (neutrophil count <500/micro L, platelet count <20,000/micro L) developed in fourpatients (10.3%). CONCLUSION: DLI was the effective salvage therapy for relapsed CML after allogeneic HSCT, whereas limited effects were shown for AML and ALL with durable remission in only a few patients.


Assuntos
Humanos , Medula Óssea , Diagnóstico , Intervalo Livre de Doença , Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Células-Tronco Hematopoéticas , Leucemia , Linfócitos , Contagem de Plaquetas , Estudos Retrospectivos , Terapia de Salvação , Seul , Doadores de Tecidos
17.
Korean Journal of Medicine ; : 656-660, 2007.
Artigo em Coreano | WPRIM | ID: wpr-112183

RESUMO

Kimura's disease is a rare chronic inflammatory disease of the lymphoid organs and this presents as painless nodules in the head and neck area. It occurs endemically in the Far East Asia and sporadically in the West. Its exact etiology is unknown and it has no potential to transform into malignant disease. However, it is often confused with malignant disease such as malignant lymphoma. We report a case of NK/T cell lymphoma patient who had Kimura's disease in the right thigh 3 years ago. She then suffered from sudden dyspnea. Radiological studies revealed multiple small nodules scattered in both lung fields. NK/T cell lymphoma was diagnosed by open lung biopsy. In addition, Epstein-Barr virus (EBV) was detected in the previous site of Kimura's disease and lung nodule by molecular pathologic examination. To the best of our knowledge, this is the first case of NK/T cell lymphoma complicating Kimura's disease with coexisting chronic EBV infection.


Assuntos
Humanos , Ásia , Biópsia , Dispneia , Infecções por Vírus Epstein-Barr , Ásia Oriental , Seguimentos , Cabeça , Herpesvirus Humano 4 , Pulmão , Linfoma , Pescoço , Coxa da Perna
18.
Journal of Korean Medical Science ; : 227-234, 2007.
Artigo em Inglês | WPRIM | ID: wpr-148960

RESUMO

We compared the outcomes of allogeneic hematopoietic stem cell transplantation using reduced intensity and myeloablative conditioning for the treatment of patients with advanced hematological malignancies. A total of 75 adult patients received transplants from human leukocyte antigen-matched donors, coupled with either reduced intensity (n=40; fludarabine/melphalan, 28; fludarabine/cyclophosphamide, 12) or myeloablative conditioning (n=35, busufan/cyclophosphamide). The patients receiving reduced intensity conditioning were elderly, or exhibited contraindications for myeloablative conditioning. Neutrophil and platelet engraftment occurred more rapidly in the reduced intensity group (median, 9 days vs. 18 days in the myeloablative group, p or =grade II) occurred at comparable frequencies in both groups, while the incidence of hepatic veno-occlusive disease was lower in the reduced intensity group (3% vs. 20% in the myeloablative group, p=0.02). The overall 1-yr survival rates of the reduced intensity and myeloablative group patients were 44% and 15%, respectively (p=0.16). The results of present study indicate that patients with advanced hematological malignancies, even the elderly and those with major organ dysfunctions, might benefit from reduced intensity transplantation.


Assuntos
Pessoa de Meia-Idade , Masculino , Humanos , Feminino , Idoso , Adulto , Adolescente , Vidarabina/administração & dosagem , Resultado do Tratamento , Transplante Homólogo/métodos , Condicionamento Pré-Transplante/métodos , Agonistas Mieloablativos/administração & dosagem , Cooperação Internacional , Transplante de Células-Tronco Hematopoéticas/métodos , Neoplasias Hematológicas/terapia , Bussulfano/administração & dosagem
19.
Korean Journal of Medicine ; : 196-206, 2006.
Artigo em Coreano | WPRIM | ID: wpr-190597

RESUMO

BACKGROUND: Acute myelogenous leukemia (AML) is frequently encountered in elderly patients whereas intensive chemotherapy yield lower rate of complete remission (CR) and survival than young patients. This study was aimed to review the clinical features and treatment outcomes of elderly patients (>or=60) with AML. METHODS: We respectively reviewed the clinical features, laboratory findings and outcomes of treatment from the medical records of 115 patients with the elderly AML (>or=60), admitted in Seoul National University Hospital, between Jan.1995 and Dec.2004. RESULTS: Their median age was 66 (60~86) years with male predominance (M:F=68:47). Complete response rate in patients with conventional chemotherapy was 66.7% (42 of 63 patients; 95% CI 50.2~78.4). Median overall survival (OS) was 5.2 months with clinical benefit in the conventional chemotherapy group, compared to supportive or palliative group (11.5 vs 0.9months; p<0.0001). In between two age groups, the sixties (n=69) showed higher CR rate (69.0 vs 61.9%; p=0.9) and longer median overall survival (7.0 vs 4.4months; p=0.8) than patients group of the seventies (n=38) but without statistical significance. CONCLUSIONS: Conventional induction chemotherapy improved survival rate than palliative or supportive treatment.


Assuntos
Idoso , Humanos , Masculino , Tratamento Farmacológico , Quimioterapia de Indução , Leucemia Mieloide Aguda , Prontuários Médicos , Prognóstico , Estudos Retrospectivos , Seul , Taxa de Sobrevida
20.
Korean Journal of Hematology ; : 92-98, 2006.
Artigo em Inglês | WPRIM | ID: wpr-720237

RESUMO

BACKGROUND: The use of non-myeloablative stem cell transplantation (NST) has recently been increasing for treating the patients who cannot tolerate ablative hematopoietic stem cell transplantation (HSCT). Although graft-versus-host disease (GVHD) is one of the greatest problems in HSCT, the clinical effect of GVHD following NST is not clear. We undertook this study to evaluate the clinical manifestations of GVHD and the outcomes after NST. METHODS: From October 2000 to October 2004, 61 patients underwent NST with a fludarabine-based conditioning regimen. The cumulative incidence of GVHD and the survival rates were obtained from the Kaplan-Meier curves. RESULTS: With a median follow-up of 195 days, the estimate for overall three-year survival was 32%. The cumulative incidences of grades II~IV acute GVHD and chronic GVHD were 33% (18/53) and 78% (29/37), respectively. The response rates for acute and chronic GVHD were 33% and 89%, respectively. The survival rates of patients with acute and chronic GVHD were 27% and 89%, respectively. The median survival time was 6.5 months CONCLUSION: The incidence of GVHD after NST did not differ from that after ablative HSCT. This study suggests that the aggressive treatment of acute GVHD should be considered to improve the overall survival after NST.


Assuntos
Humanos , Seguimentos , Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Células-Tronco Hematopoéticas , Incidência , Transplante de Células-Tronco , Taxa de Sobrevida
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