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1.
Acta Haematol ; 142(3): 162-170, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31091521

RESUMO

Aplastic anemia (AA) is a hematologic disease characterized by pancytopenia and hypocellular bone marrow, potentially leading to chronic anemia, hemorrhage, and infection. The China Aplastic Anemia Committee and British Committee for Standards in Haematology guidelines recommend hematopoietic stem-cell transplantation (HSCT) or immunosuppressive therapy (IST) comprising antithymocyte globulin (ATG) with cyclosporine (CsA) as initial treatment for AA patients. With limited epidemiological data on the clinical management of AA in Asia, a prospective cohort registry study involving 22 AA treatment centers in China was conducted to describe the disease characteristics of newly diagnosed AA patients and investigate real-world treatment patterns and patient outcomes. Of 340 AA patients, 72.9, 12.6, and 3.5% were receiving IST, traditional Chinese medicine, and HSCT, respectively, at baseline; only 22.2% of IST-treated patients received guideline-recommended ATG with CsA initially. Almost all patients received supportive care (95.6%) as blood transfusion (97.8%), antibiotics (63.7%), and/or hematopoietic growth factors (58.2%). Overall, 64.8% achieved a partial or complete response, and 0.9% experienced relapse. No new safety concerns were identified; serious adverse events were largely unrelated to the treatment regimen. These results demonstrate the need to identify and minimize treatment barriers to standardize and align AA management in China with treatment guideline recommendations and further improve patient outcomes.

2.
Pediatr Res ; 86(3): 360-364, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31112993

RESUMO

BACKGROUND: In severe aplastic anemia (SAA), predictive markers of response to immunosuppressive therapy (IST) of porcine antilymphocyte globulin (pALG) have not been well defined. We investigated whether clinical and laboratory findings before treatment could predict response in a pediatric cohort. METHODS: In this study, we included 70 newly diagnosed SAA children and treated them with pALG. The response rate was documented during follow-up. The log-rank test compared response rates between the potential predictive factors. RESULTS: The response rate was 57.1% at 24 months follow-up. In log-rank test, mild disease severity was the most significant predictive marker of better response (P < 0.001); SAA patients with higher absolute reticulocyte count (ARC) and platelet level showed a higher response rate (both P < 0.001). Although insignificantly, elderly children and male sex show better response rate after treatment. The response rate worsened when the time interval before IST was more than 60 days. CONCLUSION: Modified IST with pALG was suitable for SAA children, and favorable response correlates with mild disease severity was identified. ARC and platelet status also appeared to be a reproducible prognostic model for response rate. IST should be started as soon as possible, given that the response rate worsens as the interval between diagnosis and treatment increases.

3.
Ultrastruct Pathol ; 42(4): 350-357, 2018 Jul-Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29913101

RESUMO

Sixteen patients with mild anemia and hemolysis were difficult to be classified into any known category based on laboratory examinations and light microscopy. To make a definite diagnosis and investigate the pathomechanism, ultrastructural study was performed on erythroid cells from 16 patients. Transmission electron microscopy demonstrated a series of alterations of cytoplasm, including cytoplasm sequestration, membranous transformation, and degeneration in erythroblasts and reticulocytes at different stages. The affected erythroblasts were usually complicated with chromatin condensation, karyorrhexis, nuclear membrane lysis, and megaloblastic changes. The reticulocytes with the cytoplasm alterations had a huge size from 10 um to 15 um in diameter. The membranous cytoplasm degeneration revealed a unique pathomechanism of dyserythropoiesis and ineffective erythropoiesis in 16 patients with anemia, and suggested a novel anemia category though more details remained to be investigated.


Assuntos
Anemia/patologia , Membrana Celular/ultraestrutura , Eritroblastos/ultraestrutura , Reticulócitos/ultraestrutura , Adulto , Idoso , Medula Óssea/ultraestrutura , Núcleo Celular/ultraestrutura , Citoplasma/ultraestrutura , Eritrócitos/ultraestrutura , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
4.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 22(3): 612-6, 2014 Jun.
Artigo em Chinês | MEDLINE | ID: mdl-24989263

RESUMO

This study was aimed to explore the role and mechanism of IFN-γ in the regulation of hemopoiesis in mice. Murine IFN-γ fragment was amplified from murine splenic cells with RT-PCR and plasmid pCDH1-mIFN-γ-EF1-copGFP (pCDH-mIFN-γ-GFP) was constructed. Plasmids pCDH-mIFN-γ-GFP and pCDH1-EF1-copGFP (pCDH-GFP) together with packaging plasmids pPACK-A, pPACK-B and pPACK-C were respectively transfected into 293T cells by using a method of calcium phosphate precipitation to produce lentivirus. Bone marrow mononuclear cells (BMMNC) from male C57BL/6J mice were transfected with the lentiviral vector pCDH expressing mIFN-γ and green fluorescent protein (GFP). The cells were cultured in M3434 semi-solid medium for colony formation assay and transplanted into lethally-irradiated mice through caudal vein injection, and the peripheral blood cell counts and GFP were monitored regularly after transplantation. The results showed that lentiviral vector pCDH-mIFN-γ-GFP was constructed successfully and 293T cells transfected with mIFN-γ secreted mIFN-γ. Transfection of mIFN-γ into BMMNC decreased colony formation, colony number of the mIFN-γ group was significantly less than that of the control group. The recovering of circulating blood cell parameters in mIFN-γ transplantation group was significantly later than control group. GFP positive cells could be detected in the peripheral blood at 8 weeks after transplantation. It is concluded that mIFN-γ may inhibit the colony-forming capacity of transduced BMMNC and delay the hematopoietic reconstitution.


Assuntos
Células da Medula Óssea/efeitos dos fármacos , Hematopoese/efeitos dos fármacos , Interferon gama/farmacologia , Animais , Células da Medula Óssea/citologia , Linhagem Celular , Vetores Genéticos , Hematopoese/genética , Interferon gama/genética , Lentivirus/genética , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Plasmídeos , Transfecção
5.
Stem Cell Investig ; 1: 19, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-27358865

RESUMO

BACKGROUND: Fanconi anemia (FA) is a heterogeneous genetic disorder characterized by a progressive bone marrow aplasia, chromosomal instability, and acquisition of malignancies. Successful hematopoietic cell transplantation (HCT) for FA patients is challenging due to hypersensitivity to DNA alkylating agents and irradiation of FA patients. Early mobilization of autologous stem cells from the bone marrow has been thought to be ideal prior to the onset of bone marrow failure, which often occurs during childhood. However, the markedly decreased response of FA hematopoietic stem cells to granulocyte colony-stimulating factor (G-CSF) is circumventive of this autologous HCT approach. To-date, the mechanism for defective stem cell mobilization in G-CSF treated FA patients remains unclear. METHODS: Fancg heterozygous (Fancg (+/-)) mice utilized in these studies. Student's t-test and one-way ANOVA were used to evaluate statistical differences between WT and Fancg (-/-) cells. Statistical significance was defined as P values less than 0.05. RESULTS: Fancg deficient (Fancg (-/-)) mesenchymal stem/progenitor cells (MSPCs) produce significant lower levels of KC, an interleukin-8 (IL-8) related chemoattractant protein in rodents, as compared to wild type cells. Combinatorial administration of KC and G-CSF significantly increased the mobilization of hematopoietic stem/progenitor cells (HSPCs) in Fancg (-/-) mice. CONCLUSIONS: In summary, our results suggest that KC/IL-8 could be proved useful in the synergistic mobilization of FA HSPCs in combination with G-CSF.

6.
Zhonghua Xue Ye Xue Za Zhi ; 34(8): 709-13, 2013 Aug.
Artigo em Chinês | MEDLINE | ID: mdl-23978026

RESUMO

OBJECTIVE: To evaluate the value of serum soluble transferrin receptor (sTfR) concentration in predicting early response to immunosuppressive therapy (IST) in severe aplastic anemia (SAA). METHODS: Clinical data and hematologic responses of 140 SAA patients treated with rabbit antithymocyte globulin (rATG) combination with cyclosporine in our hospital were retrospectively analyzed. Correlation of pre-IST baseline of sTfR and IST responses was statistically analyzed and receiver operating characteristic (ROC) curve was used to estimate the sensitivity and specificity of sTfR in prediction of early responses. RESULTS: Serum concentration of sTfR in very SAA (VSAA) patients were significantly lower than SAA and transfusion dependent non-SAA cases (P=0.001). The responders, especially at 3 months, had significantly higher pre- IST baseline of sTfR [median, 0.89 (range, 0.21-2.42) mg/L] than that [median, 0.58 (range, 0.13-1.88) mg/L] of non-responders (P=0.005). The cutoff level of 0.91 mg/L and 0.88 mg/L for predicting responses at 3 and 6 months were established based on the ROC curve, with the degree of accuracy of 65.0% and 60.7% respectively. Multivariate analysis showed that pre-IST baseline of sTfR was the independent factor of predicting response at 3 months (P=0.007) and at 6 months (P=0.021). CONCLUSION: As a indicator of bone marrow failure severity, sTfR could predict early response to IST therapy in aplastic anemia.


Assuntos
Anemia Aplástica/terapia , Imunossupressão , Receptores da Transferrina/uso terapêutico , Adolescente , Adulto , Idoso , Soro Antilinfocitário/uso terapêutico , Criança , Pré-Escolar , Ciclosporina/uso terapêutico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Receptores da Transferrina/imunologia , Estudos Retrospectivos , Sensibilidade e Especificidade , Resultado do Tratamento , Adulto Jovem
7.
Zhonghua Xue Ye Xue Za Zhi ; 34(6): 532-5, 2013 Jun.
Artigo em Chinês | MEDLINE | ID: mdl-23827114

RESUMO

OBJECTIVE: To investigate the clinical features and therapeutic method for severe aplastic anemia (SAA) associated with ß-thalassemia, and to improve the recognition of the disease. METHODS: One patient hospitalized for pancytopenia was reported and the related literatures were reviewed. RESULTS: A 14-years old girl who presented with anemia from her childhood was hospitalized for acute onset of pancytopenia. Routine blood test showed that WBC count was 1.28×109/L, hemoglobin 65 g/L, platelet count 18×109/L, reticulocyte count 2×109/L, neutrophil count 0.03×109/L and mean corpuscular volume 59.6 fl, respectively. Both bone marrow aspiration and biopsy showed hypoplasia. Her red blood cells presented as microcytic hypochromic and target erythrocytes were common on peripheral blood smear. DNA analysis of the patient and her mother showed exon 17 heterozygous ß-thalassemia (c.52 A>T). A diagnosis of SAA associated with ß-thalassemia was clarified and high-dose cyclophosphamide (HD-CTX, 1.2 g/d×4 d) plus cyclosporine were offeved, which eventually led to a complete hematologic remission 12 months later. CONCLUSION: This was the first report of SAA associated with ß-thalassemia, and the regimen of HD-CTX led to a complete hematologic remission.


Assuntos
Anemia Aplástica/tratamento farmacológico , Ciclofosfamida/administração & dosagem , Imunossupressores/administração & dosagem , Talassemia beta/tratamento farmacológico , Adolescente , Anemia Aplástica/complicações , Ciclofosfamida/uso terapêutico , Feminino , Humanos , Imunossupressores/uso terapêutico , Talassemia beta/complicações
8.
Zhonghua Xue Ye Xue Za Zhi ; 34(6): 536-9, 2013 Jun.
Artigo em Chinês | MEDLINE | ID: mdl-23827115

RESUMO

OBJECTIVE: To investigate the clinical and laboratory features of 2 cases of pure red cell aplasia (PRCA) with thymoma/T-cell large granular lymphocyte leukemia (T-LGLL), and to improve the recognition of the disease and the role of lymphocyte in its mechanism. METHODS: Two cases of PRCA with thymoma/T-LGLL were reported and the related literatures were reviewed. RESULTS: Case 1 was a 63-years old male with hemoglobin level of 54 g/L at admission. Case 2 was a 52-years old female with hemoglobin level of 79 g/L at admission. They were both diagnosed as PRCA with thymoma before admission to our hospital and had no benefit from their thymectomy. Further examinations in our hospital showed that CD3⁺CD4⁻CD8⁺CD57⁺ large granular lymphocytes amplified with clonal TCR rearrangement in their peripheral blood. The diagnosis of PRCA with thymoma/T-LGLL was clarified. Case 1 did not respond to any of the frontline therapies while case 2 responded completely to cyclosporine. CONCLUSION: Both thymoma and T-LGLL could be the cause of secondary PRCA, lymphocyte proliferation may play critical role in the pathogenesis.


Assuntos
Leucemia Linfocítica Granular Grande/complicações , Aplasia Pura de Série Vermelha/complicações , Timoma/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
9.
Ultrastruct Pathol ; 37(2): 93-101, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-23573889

RESUMO

OBJECTIVE: To describe characteristics of monocytes and histiocytes in the bone marrow of patients with a confirmed and suspected diagnosis of reactive histiocytosis. METHODS: 14 patients with a confident diagnosis of reactive histiocytosis or with a suspected diagnosis were inpatients at the Tianjin Blood Diseases Hospital between 2008 and 2012. Nucleated cells from bone marrow were observed by light microscopy - morphologically and immunohistochemically for histiocyte antigens - and ultrastructurally by transmission electron microscopy. RESULTS: Monocytes, atypical histiocytes, macrophages, hemophagocytes, reticular cells and dendritic cells were significantly increased in 9, 9, 5, 3, 3 and 2, respectively, of the 14 cases. Atypical histiocytes expressed some morphological characteristics of promonocytes. CONCLUSION: Monocytes, atypical histiocytes, macrophages, hemophagocytes, reticular cells and dendritic cells were increased in different relative degrees in patients with bone marrow reactive histiocytosis or suspected reactive histiocytosis. The increase in numbers of monocytes, atypical histiocytes and macrophages was a particularly significant feature. It is argued that atypical histiocytes with immature monocyte features might be precursors of hemophagocytes, reticular cells or dendritic cells.


Assuntos
Células da Medula Óssea/ultraestrutura , Medula Óssea/ultraestrutura , Histiócitos/ultraestrutura , Histiocitose de Células não Langerhans/patologia , Monócitos/ultraestrutura , Adolescente , Adulto , Idoso , Antígenos de Diferenciação/metabolismo , Medula Óssea/metabolismo , Células da Medula Óssea/metabolismo , Exame de Medula Óssea , Contagem de Células , Pré-Escolar , Células Dendríticas/metabolismo , Células Dendríticas/ultraestrutura , Feminino , Fibroblastos/metabolismo , Fibroblastos/ultraestrutura , Histiócitos/metabolismo , Humanos , Lactente , Masculino , Microscopia Eletrônica de Transmissão , Monócitos/metabolismo , Fagócitos/metabolismo , Fagócitos/ultraestrutura , Reticulócitos/metabolismo , Reticulócitos/ultraestrutura , Adulto Jovem
10.
Zhonghua Xue Ye Xue Za Zhi ; 33(7): 566-9, 2012 Jul.
Artigo em Chinês | MEDLINE | ID: mdl-22967420

RESUMO

OBJECTIVE: To study the clinical characteristics and antimicrobial resistance of bloodstream infections caused by Gram positive bacteria, so as to provide reference for the rational use of antimicrobial agent. METHODS: One hundred and eight patients with bloodstream infections of Gram positive bacteria in our hospital from January 2009 to December 2009 were retrospectively reviewed. The clinical manifestations, pathogen types and antimicrobial susceptibility results of pathogens isolated from bloodstream were analyzed. RESULTS: All patients had fever and 31.89% with rigor, 22.41% of the patients had no local infection lesions, 77.59% had clear infection lesions, including oral infections, respiratory tract infections and soft tissue infections. The pathogen testing showed that 12.82% were staphylococci aureus, 50.42% coagulase-negative staphylococci, 24.8% streptococci, 9.4% enterococci and 2.56% Listeria monocytogenes. Antibiotics resistance of staphylococcus and enterococci in our hospital was severe. The percentage of methicillin-resistant staphylococcus aureus in this investigation was 68.92%. The resistant rates of methicillin-resistant coagulase-negative staphylococci (MRCNS) to the most antimicrobial agents were higher than that methicillin-sensitive coagulase-negative staphylococci. One strain of MRCNS was found resistant to teicoplanin and linezolid, and 1 strain of enterococci resistant to teicoplanin and linezolid. CONCLUSION: Gram positive bacteria shows serious drug resistance, but still keeps highly sensitive to vancomycin, linezolid, teicoplanin and quinupristin/dalfopristin.


Assuntos
Farmacorresistência Bacteriana , Infecções por Bactérias Gram-Positivas/diagnóstico , Infecções por Bactérias Gram-Positivas/microbiologia , Doenças Hematológicas/microbiologia , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Feminino , Bactérias Gram-Positivas/efeitos dos fármacos , Bactérias Gram-Positivas/isolamento & purificação , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
11.
Zhonghua Xue Ye Xue Za Zhi ; 33(4): 270-3, 2012 Apr.
Artigo em Chinês | MEDLINE | ID: mdl-22781715

RESUMO

OBJECTIVE: To investigate the clinical and laboratory features of congenital dyserythropoietic anemia type II (CDA-II) in order to improve the recognition of the disease. METHODS: A case of CDA-II was reported and the related literatures were reviewed. RESULTS: The 32-years old female presented with moderate anemia, jaundice and hepatosplenomegaly from her childhood and was misdiagnosed as hereditary spherocytosis for a long time. There were no increased reticulocytes in the peripheral blood and her bone marrow showed erythroid hyperplasia with 43% of binucleated erythroblasts. Electron microscopy examination revealed stretches of double membrane lining the inner surface of the erythroblast cell membrane. CONCLUSIONS: CDA-II is a rare congenital anemia characterized by ineffective erythropoiesis with unique laboratory features, and is relatively easy to be misdiagnosed. It is necessary to improve the awareness of CDA-II, and to set-up its responsible gene analysis, i.e., CDAN2 gene and SEC23B gene detection.


Assuntos
Anemia Diseritropoética Congênita/diagnóstico , Anemia Diseritropoética Congênita/genética , Adulto , Feminino , Humanos , Proteínas de Transporte Vesicular/genética
14.
Zhonghua Xue Ye Xue Za Zhi ; 32(4): 259-64, 2011 Apr.
Artigo em Chinês | MEDLINE | ID: mdl-21569710

RESUMO

OBJECTIVE: To investigate the implications of erythroblasts periodic acid-Schiff (PAS) stain for myelodysplastic syndromes (MDS) dyserythropoiesis, diagnosis and differential diagnosis. METHODS: PAS stain of bone marrow (BM) erythroblasts in 406 MDS patients, 207 non-severe aplastic anemia (NSAA), 144 immune thrombocytopenic purpura (ITP), 67 megaloblastic anemia (MegA), 76 iron deficiency anemia (IDA), 50 paroxysmal nocturnal hemoglobinuria (PNH), and 50 acute erythroid leukemia (AEL) as well as some related laboratory parameters in MDS patients were analyzed retrospectively. RESULTS: PAS-positive detection rate was significantly higher in MDS (53.0%) than in NSAA (14.5%), ITP (27.1%) and PNH (16.0%), but was significantly lower in MDS than in AEL (84.0%) (all P = 0.000). There was no significant difference in PAS-positive detection between MDS and MegA (46.3%), or MDS and IDA (40.8%) (P = 0.310, 0.052, respectively). Erythroblasts PAS-positive rate (Median, M = 1%) and PAS-positive scores (M' = 2) was significantly lower in MDS than in AEL (M = 8%; M' = 17), and significantly higher than in NSAA (M = 0%; M' = 0), ITP (M = 0%; M' = 0), PNH (M = 0%; M' = 0), MegA (M = 0%; M' = 0), and IDA (M = 0%; M' = 0) (all P < 0.05). The cut-off value of PAS-positive rate and score for distinguishing MDS from the other groups except AEL were 0.5% and 0.5, with a sensitivity and specificity of 60.8% and 74.4%, respectively. For MDS patients, the percentage of BM erythroid cells was significantly higher in PAS-positive group than in PAS-negative group (P < 0.05), and so were megakaryocyte count, lymphocyte-like micromegakaryocytes count and percentage of micromegakaryocyte (P = 0.002, 0.000, 0.000, respectively). HGB, MCV, MCH and MCHC were significantly lower in PAS-positive group (all P < 0.05), and so was the neutrophil alkaling phosphatase (NALP) (P = 0.000). PAS-positive detection rate, positive rate and score were higher in MDS patients with abnormal karyotype than with normal karyotype, and were also higher in IPSS high/intermediate-risk 2 group than in low/intermidiate-risk 1 group. CONCLUSION: The positive reaction of erythroblasts PAS stain is an indicator of dyserythropoiesis. It is helpful to the diagnosis of MDS patients.


Assuntos
Eritroblastos , Síndromes Mielodisplásicas/diagnóstico , Reação do Ácido Periódico de Schiff , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sensibilidade e Especificidade , Coloração e Rotulagem , Adulto Jovem
16.
Zhonghua Xue Ye Xue Za Zhi ; 32(1): 38-42, 2011 Jan.
Artigo em Chinês | MEDLINE | ID: mdl-21429400

RESUMO

OBJECTIVE: To analyze the efficacy and side-effects of combination of rabbit antithymocyte globulin (ATG) and cyclosporine A (CsA) as the first-line immunosuppressive therapy (IST) for adult severe aplastic anemia (SAA) patients. METHODS: Adult SAA or very severe aplastic anemia (VSAA) patients treated with rabbit ATG + CsA as first line therapy in our hospital from 2003 to 2008 were retrospectively analysed and the therapeutic response relevant factors were analysed. RESULTS: Seventy-nine patients were enrolled. Of all these patients, 6 died within 3 months after IST. The overall response rate was 82.2% and the median time to transfusion independent was 60 days. The therapeutic response rate in 32 SAA patients (100%) was significantly higher than that in 41 VSAA cases (68.3%) (P = 0.001). Patients with neutrophil response to G-CSF treatment had a higher IST response rate than those without response to G-CSF (100% vs 67.5%, P = 0.001). Sixty-one patients (77.2%) occurred serum sickness reaction. Three patients relapsed and two developed clonal hematological abnormalities after IST. The 3-year overall survival for all the patients was 88.9%. CONCLUSIONS: Rabbit ATG in combination with CsA as first-line IST for adult SAA can lead to excellent treatment outcomes with minor adverse effects.


Assuntos
Anemia Aplástica/tratamento farmacológico , Soro Antilinfocitário/uso terapêutico , Ciclosporina/uso terapêutico , Imunossupressores/uso terapêutico , Adolescente , Adulto , Animais , Quimioterapia Combinada , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Coelhos , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
19.
Zhonghua Xue Ye Xue Za Zhi ; 32(11): 766-71, 2011 Nov.
Artigo em Chinês | MEDLINE | ID: mdl-22339914

RESUMO

OBJECTIVE: To evaluate the effects of cyclosporine A (CsA) whole-blood concentration on the early response to immunosuppressive therapy (IST) in severe and very severe aplastic anemia (SAA/VSAA). METHODS: Ninety SAA/VSAA patients treated with rabbit antithymocyte globulin (ATG) plus CsA as first line therapy in our hospital were retrospectively analysed. CsA levels between the response group and non-response group, and response rates of patients with variant CsA levels were compared respectively. RESULTS: (1) There was no significant difference in the beginning unmodified CsA blood concentration between IST responded and non-responded SAA/VSAA patients. The beginning unmodified C(0) 133.91 ug/L in IST 2-month responders was higher than that of 49.9 ug/L in non-responded SAA patients (P = 0.009); (2) The mean CsA C(0) and C(2) levels during the third month following IST were significantly different in responders and non-responders(197.52 µg/L vs 161.49 µg/L, P = 0.024, and 738.76 µg/L vs 615.46 µg/L, P = 0.009), and no significant difference in other periods of IST (P > 0.05); (3) The response rate (87.5%) was significantly higher in patients with CsA C(0) ≥ 200µg/L the third month following IST than those of 55.6% in patients with CsA C(0) 150 - 200 µg/L (P = 0.023) and 59.3% in patients with CsA C(0) < 150 µg/L (P = 0.046), respectively. The response rate was significantly higher of C(2) ≥ 700 µg/L group than that of C(2) < 700 µg/L group (80.5%vs 55.3%, P = 0.012). CONCLUSIONS: The CsA concentration related to the early IST response. The third month CsA concentrations was the most important for the response and maintaining CsA levels with C(0) ≥ 200 µg/L and C(2) ≥ 700 µg/L may improve the response to IST in SAA/VSAA.


Assuntos
Anemia Aplástica/sangue , Anemia Aplástica/terapia , Ciclosporina/sangue , Imunossupressores/uso terapêutico , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
20.
Zhonghua Xue Ye Xue Za Zhi ; 31(7): 475-7, 2010 Jul.
Artigo em Chinês | MEDLINE | ID: mdl-21122403

RESUMO

OBJECTIVE: To evaluate the initial clinical characteristics, the response to treatment, and the outcome in adult patients with Evans syndrome. METHODS: The clinical data of 84 adult patients (20 males, 64 females) with Evans syndrome diagnosed at our center between 1984 and 2007 were retrospectively analyzed. RESULTS: The patients were followed up for a median duration of 17.5 (0.03 - 140) months. All the patients initially received intravenous steroids with or without intravenous immunoglobulin (IVIG). Forty-seven patients were treated with corticosteroids alone initially. Complete remission (CR) and partial remission (PR) were achieved in 38 of the patients, but 92.1% of them relapsed during a median follow-up of 12 months. Twenty-eight patients who were resistant to corticosteroids therapy or with severe bleeding were subsequently administered immunosupressive agents. CR and PR were obtained in 89.3% of them. Within a median follow-up of 8 months, 84% of these patients relapsed. CONCLUSIONS: Evans syndrome is a chronic and easy to recurrent disease, which is often refractory to conventional therapy. Treatment with combination agents might be a useful therapeutic approach to the patients.


Assuntos
Anemia Hemolítica Autoimune , Indução de Remissão , Corticosteroides , Adulto , Seguimentos , Humanos , Estudos Retrospectivos , Resultado do Tratamento
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