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1.
Article in Chinese | WPRIM | ID: wpr-880173

ABSTRACT

OBJECTIVE@#To investigate the clinical characteristics and risk factors of cytomegalovirus (CMV) infection after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in patients with severe aplastic anemia (SAA).@*METHODS@#Clinical data from 270 SAA patients with allo-HSCT were retrospectively analyzed, including 108 sib congruence patients and 162 substitute donors (68 unrelated donor congruence patients and 94 related haploid patients). Different pretreatment schemes were selected for different transplantation modes. The HLA-identical sibling and haploid grafts were all bone marrow and peripheral blood stem cells, and the grafts from unrelated donors were peripheral blood stem cells. After granulocyte implantation, blood CMV-DNA was regularly monitored. Flow cytometry was also used to determine the absolute number of CD3@*RESULTS@#CMV infection occurred in 229 of 270 patients with an incidence of 84.8%. Among them, 18 patients developed giant cell disease. Univariate analysis showed that alternative donors (unrelated total and haploid donors), mycophenolate mofetil and acute graft-versus-host disease were statistically significantly associated with CMV infection (P<0.05). Multivariate analysis showed that alternative donors were associated with CMV infection. The recovery of CD3@*CONCLUSION@#After allo-HSCT, substitute donors are more easily to develop CMV infection than full-sibling donors, and the reconstruction of immune function is delayed after transplantation.


Subject(s)
Anemia, Aplastic , Cytomegalovirus Infections , Graft vs Host Disease , Hematopoietic Stem Cell Transplantation , Humans , Retrospective Studies
2.
Article in Chinese | WPRIM | ID: wpr-880113

ABSTRACT

METHODS@#To establish the acquired aplastic anemia mouse model through the X-ray irradiation in combination with lymphocytes injection. AA Group: the purified Pan T lymphocytes from the spleen of C57BL/6J mice were enriched and injected to the mice through tail vein(5×10@*RESULTS@#Compared with 4, 5 Gy irradiated mice in AA groups, the survival time of 3 Gy irradiated AA groups was significantly prolonged. 3, 4 and 5 Gy X-ray irradiation combined with Pan T lymphocyte injection could successfully induced severe reduction of red blood cells, blood neutrophils, and platelets, severe reduction of bone marrow nucleated cells, severe bone marrow hematopoietic failure, and the significant expansion of T lymphocytes ratio in the bone marrow. CD4@*CONCLUSION@#3, 4 and 5 Gy X-ray irradiation combined with 5×10


Subject(s)
Anemia, Aplastic , Animals , Bone Marrow , Bone Marrow Cells , CD8-Positive T-Lymphocytes , Humans , Mice , Mice, Inbred C57BL
3.
Article in Chinese | WPRIM | ID: wpr-880055

ABSTRACT

OBJECTIVE@#To explore the relationship between the change of lymphocyte subsets before and after immunosuppressive therapy (IST) with disease severity of severe aplastic anemia (SAA) and hematologic response to IST.@*METHODS@#The clinical data of 94 patients with SAA/VSAA treated by r-ATG and CsA in our hospital from December 2009 to October 2011 was analyzed retrospectively. Among them, 26 patients who had sequential data of lymphocyte subsets and cytokines before and after treatment were enrolled. The relationship between lymphocyte subsets, cytokine level before IST and disease severity, as well as the relationship between changes if lymphocyte subsets, changes of cytokine and the HR after IST for 6 months was analyzed.@*RESULTS@#There were no statistical differences in the ratio and absolute count of lymphocyte, the ratio and absolute count of each lymphocyte subsets, including CD3@*CONCLUSION@#The hematopoietic recovery and early hematologic remission may be affected by the intensity of immune suppression reflected from the changes of lymphocyte subsets and the immune reconstruction reflected from the recovery of lymphocyte subsets. The immune reconstruction is most significant within 3 months after IST.


Subject(s)
Anemia, Aplastic , Humans , Immunosuppression , Immunosuppressive Agents/therapeutic use , Lymphocyte Subsets , Retrospective Studies
4.
Rev. cuba. hematol. inmunol. hemoter ; 36(3): e1277, jul.-set. 2020. tab, graf
Article in Spanish | LILACS, CUMED | ID: biblio-1156444

ABSTRACT

Introducción: La aplasia medular adquirida grave es una enfermedad hematológica infrecuente caracterizada por una disminución o ausencia de precursores hematopoyéticos en la médula ósea, lo cual se expresa con distintos grados de citopenias. Varios factores, infecciosos o no, pueden incidir en su origen. Su manejo es complejo y puede incluir tratamiento inmunosupresor y trasplante de progenitores hematopoyéticos alogénico. Objetivo: Demostrar la utilidad de la realización del trasplante de progenitores hematopoyéticos alogénico haploidéntico en pacientes con aplasia medular grave. Caso clínico: Paciente masculino de 21 años de edad, con antecedentes de salud, que en octubre del 2018 debutó con íctero, pancitopenia, lesiones purpúrico hemorrágicas en piel y mucosas, en el curso de una hepatitis aguda seronegativa. La biopsia de médula ósea mostró aplasia medular severa. Se inició tratamiento inmunosupresor con globulina antitimocίtica, ciclosporina A y metilprednisolona. Al cabo de los 6 meses mantenía trombocitopenia severa con necesidades transfusionales y en octubre de 2019 se decide realizar trasplante de progenitores hematopoyéticos alogénico con donante haploidéntico y empleando como tratamiento acondicionante globulina antitimocίtica, fludarabina, ciclofosfamida y bajas dosis de irradiación corporal total. En evaluación clίnica de julio de 2020 (dίa + 280 del trasplante) el paciente estaba asintomático y con parámetros hematológicos normales. Conclusiones: Se demostró que el trasplante de progenitores hematopoyéticos alogénico haploidéntico es un proceder realizable y útil en pacientes con aplasia medular grave, lo cual corrobora el beneficio clínico que puede aportar su ejecución en pacientes con esta enfermedad(AU)


Introduction: Acquired severe marrow aplasia is a rare hematological disease characterized by decrease or absence of hematopoietic precursors in bone marrow, which is expressed with different degrees of cytopenias. Several factors, infectious or not, can influence its origin. Its management is complex and may include immunosuppressive treatment and allogeneic hematopoietic stem-cell transplantation. Objective: To demonstrate the usefulness of performing haploidentical allogeneic hematopoietic stem-cell transplantation in patients with severe medullary aplasia. Clinical case: A 21-year-old male patient, with medical history, who first presented, in October 2018, with icterus, pancytopenia, as well as purpuric hemorrhagic lesions on the skin and mucosa, in the course of acute seronegative hepatitis. The bone marrow biopsy showed severe marrow aplasia. Immunosuppressive treatment was started with antithymocytic globulin, cyclosporine A, and methylprednisolone. After six months, he maintained severe thrombocytopenia under transfusion requirements and, in October 2019, the decision was to perform allogeneic hematopoietic stem-cell transplantation with a haploidentical donor and using antithymocyte globulin, fludarabine, cyclophosphamide, and low doses of total body irradiation as conditioning treatment. In the clinical assessment carried out in July 2020 (day +280 after transplantation), the patient was asymptomatic and with normal hematological parameters. Conclusions: Transplantation of haploidentic allogeneic hematopoietic progenitors was shown to be a feasible and useful procedure in patients with severe marrow aplasia, which corroborates the clinical benefit that its execution can bring in patients with this disease(AU)


Subject(s)
Humans , Male , Young Adult , Tissue Donors/ethics , Methylprednisolone/therapeutic use , Whole-Body Irradiation/methods , Microscopy, Electron, Scanning Transmission/methods , Hematologic Diseases , Hematopoietic Stem Cell Transplantation/methods , Cuba , Transplantation, Haploidentical/methods , Anemia, Aplastic/therapy , Antilymphocyte Serum
5.
Article in Chinese | WPRIM | ID: wpr-827186

ABSTRACT

OBJECTIVE@#To explore the effect of miR-335-5p/ADCY3 interaction on the lymphocyte function in the patients with aplastic anemia (AA).@*METHODS@#Blood samples were collected from 22 healthy volunteers (HC) and 50 AA patients including 38 severe AA (SAA) and 12 non-severe AA (NSAA). Peripheral blood mononuclear cells (PBMNC) were isolated. The expression of miR-335-5p and ADCY3 mRNA was detected by using RT-PCR. Negative control miR-335-5p (NC group) and miR-335-5p mimic (mimic group) were transfected to AA-PBMNC by using RNAimax reagent, respectively. The proliferative ability, activation and cytokines of CD4 T and CD8 T cells were measured by flow cytometry. Dual-luciferase reporter assay was used to verify the targeted relationship between miR-335-5p and target gene.@*RESULTS@#The expression of miR-335-5p was significantly downregulated in SAA-PBMNC and NSAA-PBMNC compared with HC-PBMNC (0.08±0.01 vs 0.74±0.10, P<0.01; 0.17±0.02 vs 0.74±0.10, P<0.01). Meanwhile, the expression of miR-335-5p in SAA-PBMNC was very statistically significantly lower than that in NSAA-PBMNC (P<0.01). Compared with NC group, upregulation of miR-335-5p in vitro could significantly inhibited the proliferation of CD4 T and CD8 T cells in AA-PBMNC (P<0.05 and P<0.05, respectively). And, upregulating miR-335-5p in AA-PBMNC could significantly inhibited the activation of CD4 and CD8 T cells (P<0.01 and P<0.01, respectively). The ratio of CD4TNFα T, CD8IFNγ+T and CD8TNFα T cell by up-regulating the expression of miR-335-5p from AA-PBMNC in vitro was also significantly lower (P<0.01, P<0.05 and P<0.05, respectively). In addition, the expression of ADCY3 was higher in AA-PBMNC than that in HC-PBMNC (1.70±0.15 vs 0.76±0.12, P<0.01). Furthermore, by means of dual-luciferase reporter assay, the luciferase activity of ADCY3'UTR wildtype could be inhibited by miR-335-5p.@*CONCLUSIONS@#The expression of miR-335-5p was significantly downregulated in AA, and that correlates with disease severity. Up-regulating miR-335-5p can correct the hyperimmune status in AA patients by targeting ADCY3. These changes may relates with the strengthen of inhibition for targeted gene ADCY3.


Subject(s)
Anemia, Aplastic , Genetics , CD8-Positive T-Lymphocytes , Humans , Leukocytes, Mononuclear , Lymphocyte Count , MicroRNAs , Genetics
6.
Article in Chinese | WPRIM | ID: wpr-827184

ABSTRACT

OBJECTIVE@#To establish a stable and rapidly rat model acquired aplastic anemia.@*METHODS@#The SD rats were exposed to Csγ-ray at 3.5 and 4.0 Gy ( 91 cGy/min), and intraperitoneally injected with CTX at 35 mg/( kg·d) and CHL at 45 mg/( kg·d) in d 4, 6 and 8 after irradiation; the WBC, platelet and reticulocyte counts in peripheral blood, the smears and nucleated cells counts of bone marrow were observed.@*RESULTS@#The levels of peripheral blood 3-lineage cells of SD rats treated with Csγ-irradiation combined with cyclophosphamide and chloramphenicol were significantly reduced, among which white blood cells, platelets and reticulocytes decreased rapidly, and the number of bone marrow nucleated cells decreased significantly; bone marrow pathological sections showed severe reduction of hematopoietic cells, and the non-hematopoietic cells such as fat cells increased, and a serve or lightly reduction of bone marrow cells were found.@*CONCLUSION@#The rat model established by Csγ-ray irradiation combined with cyclophosphamide and chloramphenicol meets the clinical characteristics of aplastic anemia, and this study provides a stable rat model for the study of new therapeutic drugs for acquired aplastic anemia.


Subject(s)
Anemia, Aplastic , Animals , Bone Marrow , Bone Marrow Cells , Cyclophosphamide , Rats , Rats, Sprague-Dawley
7.
Article in Chinese | WPRIM | ID: wpr-827182

ABSTRACT

OBJECTIVE@#To investigate the effects of different routes in placental mesenchymal stem cells (PMSC) on serum expression levels of IL-4, IL-17, TNF-α and IFN-γ in aplastic anemia (AA) rats.@*METHODS@#The rat model of aplastic anemia (AA rats) was established by 5-fluorouracil combined with busulfan. The rats was divided into four groups: control, experimental, PMSC-injected into the tail vein, and PMSC-injected into the medullary cavity. The general state of rats in each group was observed in detail before and after treatment. The serum levels of interleukin-4 (IL-4) , interleukin-17 (IL-17), tumor necrosis factor-α (TNF-α), interferon-γ (IFN-γ) were measured by enzyme-linked immunosorbent assay (ELISA) at week 1, 3 and 5 after treatment.@*RESULTS@#The serum levels of TNF-α, IFN-γ and IL-17 in the experimental group were significantly higher than those in the control group, while the level of IL-4 was significantly decreased (P<0.05). The levels of TNF-α, IFN-γ and IL-17 gradually decreased after treatment while the level of IL-4 increased. By the fifth week, the above indexes were closed to the control group (P>0.05), and the levels of TNF-α, IFN-γ and IL-17 in the group with PMSCs injected via the medullary cavity decrease more significantly than those group with PMSC injected via the tail vein, but level of IL-4 was not significantly different between two groups.@*CONCLUSION@#The level of serum hematopoietic negative regulators increase significantly, and the level of hematopoietic promoting factors decreases significantly in aplastic anemia rats. PMSC can down-regulate the level of hematopoietic negative regulators and up-regulate the level of hematopoietic promoting factors in the rats with aplastic anemia, and the inhibition of hematopoietic negative regulators by intramedullary injection is more significant than that by caudal vein injection.


Subject(s)
Anemia, Aplastic , Animals , Female , Hematopoietic Stem Cell Transplantation , Interferon-gamma , Mesenchymal Stem Cells , Placenta , Pregnancy , Rats , Tumor Necrosis Factor-alpha
8.
Article in English | WPRIM | ID: wpr-810958

ABSTRACT

BACKGROUND: This study aimed to assess the outcome of stem cell transplantation (SCT), including overall survival (OS), failure-free survival (FFS) and graft-versus-host disease (GvHD)-free/failure-free survival (GFFS), and to analyze prognostic factors in children with aplastic anemia (AA).METHODS: From 1991 to 2018, 43 allogeneic SCT recipients were enrolled in the study to investigate the demographic characteristics, survival outcomes and prognostic factors.RESULTS: With the median follow-up of 7.1 years, the estimated 10-year OS, FFS, GFFS were 86.0%, 60.5%, and 51.2%, respectively. Matched related donors (MRD, n = 28) showed better 10-year OS than unrelated donors (n = 15) (96.4% vs. 66.7%; P = 0.006). Engraftment failure was seen in 13 patients (30.2%). Donor-type aplasia was seen in 13.8% (4/29) after fludarabine (Flu)-based conditioning (Flu-group), while in 42.6% (6/14) after cyclophosphamide (Cy)-based regimen (Cy-group) (P = 0.035). Six patients died. The 10-year OS in Cy-group was 92.9% (n = 14, all MRD), while that of Flu-group was 82.1% (n = 29; P = 0.367). But Flu-group tended to have better FFS and GFFS than Cy-group, although Flu-group had less MRDs (41.4% vs. 100%; P = 0.019), and higher proportion of previous immunosuppressive treatment (IST; 62% vs. 21.4%, P = 0.012). In MRD transplants, OS was similar between Flu-group (100%, n = 14) and Cy-group (92.9%, n = 14), while FFS (100.0% vs. 42.9%; P = 0.001) and GFFS (85.7% vs. 35.7%; P = 0.006) were significantly better in Flu-group. Stem cell sources, irradiation in the conditioning, and method of GvHD prophylaxis did not significantly influence the outcome.CONCLUSION: This study reviewed SCT outcomes for pediatric AA with changes of transplant strategies over the last 25 years. The FFS and GFFS were higher in Flu-group than in Cy-group, especially in matched related transplantation. Graft failure including donor-type aplasia remains troublesome even with Flu-based conditioning. Further refinement of transplant strategies to ensure better quality-of-life should be pursued.


Subject(s)
Anemia, Aplastic , Child , Cyclophosphamide , Follow-Up Studies , Graft vs Host Disease , Humans , Methods , Stem Cell Transplantation , Stem Cells , Tissue Donors , Transplants , Unrelated Donors
11.
Rev. Assoc. Med. Bras. (1992) ; 65(5): 637-646, May 2019. graf
Article in English | LILACS | ID: biblio-1012952

ABSTRACT

SUMMARY OBJECTIVE: Aplastic anemia (AA) is an immune-mediated disease that destroys hematopoietic cells through activated T lymphocytes. B lymphocyte-mediated humoral immunity also plays an important role in the pathogenesis of AA. Regulatory B cell (Breg) subpopulation, which is defined as "B10", secretes interleukin 10 (IL-10). The objective of our experiment was to investigate whether the scale-down proportion of B10 cells in AA patients may play a key role in the pathogenesis. METHODS: A total of 38 AA patients (14 SAA patients and 24 NSAA patients) and 20 healthy control subjects were included. All subjects did not suffer from autoimmune diseases or any other diseases affecting the immune system, such as infectious diseases. Bone marrow mononuclear cells (PBMCs) were isolated and analyzed by Flow cytometry (FCM) and Immunofluorescence double-labeling assay. The relationship between the relative proportions of B10 and ProB10 and their associations to AA, as well as disease severity, were assessed by common clinical indicators and then examined. RESULTS: Our analyses revealed AA patients had significantly lower proportions of peripheral B10 and B10pro compared to healthy controls. SAA patients had a substantially lower percentage of B10 cells and B10pro cells compared to NSAA patients. In addition, B10 cells and B10pro cells were negatively correlated with absolute neutrophil counts, hemoglobin levels and platelet, and absolute reticulocyte counts in AA patients. CONCLUSIONS: The present study attempted to elucidate the potential role of the scale-down proportion of B10 cells in the pathogenesis of AA.


RESUMO OBJETIVO: A anemia aplástica (AA) é uma doença imunomediada que destrói células hematopoiéticas por meio dos linfócitos T ativados. A imunidade humoral mediada por linfócitos B também desempenha um papel importante na patogênese da AA. A subpopulação de células B reguladoras (Breg), que é definida como "B10", secreta interleucina 10 (IL-10). No experimento, investigou-se se a proporção reduzida de células B10 nos pacientes de AA pode desempenhar um papel-chave na patogênese. MÉTODOS: Um total de 38 pacientes de AA (14 pacientes de anemia aplástica grave e 24 pacientes de anemia aplástica não grave) e 20 indivíduos de controle saudáveis foram incluídos. Todos os indivíduos não sofriam de doenças autoimunes ou de quaisquer outras doenças que afetam o sistema imunológico, tais como doenças contagiosas. As células mononucleares da medula óssea (PBMCs) eram isoladas e analisadas por citometria de fluxo (FCM) e ensaio de dupla marcação por imunofluorescência. A relação entre as proporções relativas de células B10 e as células ProB10 e as suas associações à AA, assim como a gravidade da doença avaliada por indicadores clínicos comuns, foram examinadas. RESULTADOS: Nossas análises revelaram que os pacientes de AA têm proporções significativamente menores de células B10 e células ProB10 periféricas em comparação com indivíduos de controle saudáveis. Os pacientes de anemia aplástica grave tiveram uma percentagem substancialmente menor de células B10 e células B10pro em comparação com pacientes de anemia aplástica não grave. Além disso, as células B10 e B10pro foram negativamente correlacionadas com contagens absolutas de neutrófilos, níveis de hemoglobina e plaquetas e contagem de reticulócitos absolutos nos pacientes de AA. CONCLUSÕES: Além disso, o estudo presente tentou elucidar o papel imunorregulatório potencial das células B10 na patogênese da AA e fornecer uma nova estratégia para a aplicação de imunoterapia baseada na célula B para tratar a AA no futuro.


Subject(s)
Humans , Male , Female , Adolescent , Adult , Aged , Young Adult , B-Lymphocytes, Regulatory/pathology , Anemia, Aplastic/pathology , Reference Values , Severity of Illness Index , Bone Marrow Cells/cytology , Case-Control Studies , Cells, Cultured , Fluorescent Antibody Technique , Interleukin-10/analysis , Interleukin-10/metabolism , Reticulocyte Count , Antigens, CD19/analysis , Antigens, CD19/metabolism , Flow Cytometry , Anemia, Aplastic/blood , Leukocyte Count , Middle Aged , Neutrophils
12.
Article in English | WPRIM | ID: wpr-785623

ABSTRACT

Herbal products have been used for therapeutic purposes for a long time. However, many herbs can be toxic and even life-threatening. If refractory ventricular tachycardia (VT) is caused by herbal products and shows no response to conventional therapy, intravenous lipid emulsion (ILE) therapy can be considered. We report a case of herbal intoxication leading to refractory VT, which was successfully treated with ILE therapy. A 36-year-old woman with aplastic anemia presented with mental changes. She had taken an unknown herbal decoction three days before visiting the hospital. Soon after coming to the hospital, she went into cardiac arrest. Cardiopulmonary resuscitation was performed, and return of spontaneous circulation with VT was achieved. Synchronized cardioversion was then performed and amiodarone was administered. However, VT with pulse continued, so ILE therapy was attempted, which led to the resolution of VT.


Subject(s)
Adult , Amiodarone , Anemia, Aplastic , Cardiopulmonary Resuscitation , Electric Countershock , Fat Emulsions, Intravenous , Female , Heart Arrest , Herb-Drug Interactions , Humans , Tachycardia, Ventricular
13.
Article in English | WPRIM | ID: wpr-785307

ABSTRACT

The dose of CD34+ cells is known to influence the outcome of allogeneic peripheral blood stem cell (PBSC) and/or T-cell-depleted transplantation. A previous study proposed that 2×10⁶ CD34+ cells/kg is the ideal minimum dose for allogeneic transplantation, although lower doses did not preclude successful therapy. In the case we present here, CD34+ cells were collected from a matched sibling donor on the day of allogeneic hematopoietic stem cell transplantation; however, the number of cells was not sufficient for transplantation. Consequently, PBSCs were collected three additional times and were infused along with cord blood cells from the donor that were cryopreserved at birth. The cumulative dose of total nuclear cells and CD34+ cells was 15.9×10⁸ cells/kg and 0.95×10⁶ cells/kg, respectively. White blood cells from this patient were engrafted on day 12. In summary, we report successful engraftment after infusion of multiple low doses of CD34+ cells in a patient with severe aplastic anemia.


Subject(s)
Anemia, Aplastic , Cord Blood Stem Cell Transplantation , Fetal Blood , Hematopoietic Stem Cell Transplantation , Humans , Leukocytes , Parturition , Peripheral Blood Stem Cell Transplantation , Siblings , Stem Cells , Tissue Donors , Transplantation, Homologous
14.
Article in Chinese | WPRIM | ID: wpr-771925

ABSTRACT

OBJECTIVE@#To improve and establish the mouse model with aplastic anemia (AA) mediated by Cs γ-ray irradiation combined with cyclophosphamide (CTX) and chloramphenicol (CHL) injection,so as to provide a stable model for studying the pathogenesis and treatment of AA .@*METHODS@#The BALB/c mice were exposed to Cs γ-ray of 3-5 Gy(91 cGy/min) and were intraperitoneally injected with CTX of 25 mg/(kg.d) and CHL of 62.5 mg/(kg.d) at D 4,5 and 6 after irradiation; the WBC, platelet and reticulocyte counts in peripheral blood as well as the mucleated cell count in bone marrow and bone marrow smears were detected .@*RESULTS@#The 3-lineage cells in peripheral blood of BALB/c mouse model with acquired AA were rapidly reduced, especially WBC, platelet and reticulocyte counts were lowest at D 14,the 3-lineage cells in peripheral blood were still severely reduced at D 28; the nucleated cell count in bone marrow significantly dcreased,the bone marrow hyperplasia was reduced or severely reduced; the pathological sections of bone marrow showed the severe reduction of hematopoietic cells and the increased of non-hematopoietic cells such as fat cells.@*CONCLUSION@#The mouse model with acquired AA has been established by Cs γ-ray irradiation combined with CTX and CHL injection. All detection indicators of this model reach to diagnostic criteria for acquired AA,therefore this mouse model may be used as the model for study of pathogenesis and treatment of acquired AA.


Subject(s)
Anemia, Aplastic , Animals , Chloramphenicol , Cyclophosphamide , Gamma Rays , Mice , Mice, Inbred BALB C
15.
Journal of Experimental Hematology ; (6): 1215-1219, 2019.
Article in Chinese | WPRIM | ID: wpr-775739

ABSTRACT

OBJECTIVE@#To evaluate the clinical efficacy of low dose combined chemotherapy(LDCC) for patients with relapsed and refractory aplastic anemia-paroxysmal nocturnal hemoglobinuria(AA-PNH) syndrome, and to analyze the advantages of LDCC in the treatment of AA-PNH syndrome.@*METHODS@#The clinical characteristics and the curative effect of LDCC in 9 patients with relapsed and refractory AA-PNH syndrome were retrospectively analyzed. Five patients were treated with MP therapy[melphalan 2 mg/(m·d); prednisone 0.5 mg/(kg·d)], and the other 4 patients were treated with HA therapy(HHT 2 mg/d iv drip, for 5 days; Ara-C 100 mg/d iv drip, for 5 days). The changes of PNH clone, dosage of corticosteroid, hemolysis and the relapse of disease, hematological parameters and adverse reactions were compared before and after therapy. All patients were treated for 1-2 courses.@*RESULTS@#Seven out of 9 patients responded well, the dosage of corticosteroid and the bilirubin concentration decreased significantly and anemia was relieved in 7 patients (P<0.05). One patient relapsed in one year. PNH clone of 3 patients turned negative. Five patients did not rely on blood transfusion in 1 year. There was no bone marrow failure to be found in all patients.@*CONCLUSION@#The LDCC has better efficacy and safety in the treatment of patients with AA-PNH syndrome, moreover, the patients is more tolerant to LDCC, thus the LDCC may be a selection for treatment of patients with relapsed and refractory AA-PNH syndrome.


Subject(s)
Anemia, Aplastic , Anemia, Refractory , Hemoglobinuria, Paroxysmal , Hemolysis , Humans , Retrospective Studies
16.
Article in English | WPRIM | ID: wpr-741643

ABSTRACT

Hematopoiesis has a pivotal role in the maintenance of body homeostasis. Ironically, several hematological disorder caused by chemicals, drugs, and other environmental factors lead to severe bone marrow failure. Current treatments like stem cell transplantation and immunosuppression remain ineffective to ameliorate this diseases. Therefore, a newtreatment to overcome this entity is necessary, one of them by promoting the usage of medicinal plants. Thus, this study aimed to evaluate the hematopoiesis potency of S. javanica berries and leaves extracts in chloramphenicol (CMP)-induced aplastic anemia mice model. In this present study, several types of blood progenitor cell such as TER-119⁺VLA-4⁺ erythrocytes lineage, Gr-1⁺ granulocytes, and B220⁺ B-cell progenitor cells were evaluated by flow cytometry analysis. Accordingly, we revealed that S. javanica berries and leaves extracts significantly promoted TER-119⁺VLA-4⁺ erythrocytes lineage and Gr-1⁺ granulocytes after exposed by CMP. Thus, these results suggested that S. javanica berries and leaves extracts might have hematopoiesis activity in CMP-induced aplastic anemia mice model.


Subject(s)
Anemia, Aplastic , Animals , B-Lymphocytes , Bone Marrow , Chloramphenicol , Erythrocytes , Flow Cytometry , Fruit , Granulocytes , Hematopoiesis , Homeostasis , Immunosuppression , Iron , Mice , Plants, Medicinal , Sambucus , Stem Cell Transplantation , Stem Cells
17.
Safety and Health at Work ; : 347-354, 2019.
Article in English | WPRIM | ID: wpr-761366

ABSTRACT

BACKGROUND: The aim of this study was to describe the types of diseases that developed in semiconductor workers who have registered with the Korea Workers' Compensation and Welfare Service (KWCWS) and to identify potential common occupational characteristics by the type of claimed disease. METHODS: A total of 55 semiconductor workers with cancer or rare diseases who claimed to the KWCWS were compared based on their work characteristics and types of claimed diseases. Leukemia, non-Hodgkin lymphoma, and aplastic anemia were grouped into lymphohematopoietic (LHP) disorder. RESULTS: Leukemia (n = 14) and breast cancer (n = 10) were the most common complaints, followed by brain cancer (n = 6), aplastic anemia (n = 6), and non-Hodgkin lymphoma (n = 4). LHP disorders (n = 24) accounted for 43%. Sixty percent (n = 33) of registered workers (n = 55) were found to have been employed before 2000. Seventy-six percent (n = 42) of registered workers and 79% (n = 19) among the registered workers with LHP (n = 24) were found to be diagnosed at a relatively young age, ≤40 years. A total of 18 workers among the registered semiconductor workers were finally determined to deserve compensation for occupational disease by either the KWCWS (n = 10) or the administrative court (n = 8). Eleven fabrication workers who were compensated responded as having handled wafers smaller than eight inches in size. Eight among the 18 workers compensated (44 %) were found to have ever worked at etching operations. CONCLUSION: The distribution of cancer and rare diseases among registered semiconductor workers was closely related to the manufacturing era before 2005, ≤8 inches of wafer size handled, exposure to clean rooms of fabrication and chip assembly operations, and etching operations.


Subject(s)
Anemia, Aplastic , Brain Neoplasms , Breast Neoplasms , Compensation and Redress , Environment, Controlled , Korea , Leukemia , Lymphoma, Non-Hodgkin , Occupational Diseases , Rare Diseases , Semiconductors , Workers' Compensation
18.
Journal of Experimental Hematology ; (6): 1925-1932, 2019.
Article in Chinese | WPRIM | ID: wpr-781517

ABSTRACT

OBJECTIVE@#To evaluate the regulation of VEGF-Notch signaling pathway on proliferation and apoptosis of mesenchymal stem cells (MSC) in the patients with aplastic anemia (AA).@*METHODS@#The bone marrow specimens of AA patients were collected for isolation and identification of BM MSC. Westen blot was used to detect the expression of VEGF-Notch signaling pathway-related proteins (VEGF, VEGFR, Notch 1, Jagged 1, Delta-like 1 and Hes1). The VEGF (100 ng/ml) and DAPT (γ-secretase inhibitor, 10 μmol/L) were respectively added into MSC culture system in oder to activate and inhibit the signaling transduction of VEGF-Notch in BM MSC. The proliferation, apoptosis and cell cycle of MSC in AA patients were detected by CCK8 assay and flow cyfometry. The adipogenic differentiation of BM MSC was detected by oil red O staining.@*RESULTS@#The VEGF-Notch signaling pathway was significantly inhibited in AA BM tissues and AA MSC (P<0.05) detected by Western blot. The intervention of VEGF and DAPT significantly activated and inhibited VEGF-Notch signaling in AA MSC, respectively. CCK8 assay showed that VEGF intervention significantly promoted the proliferation of MSC in AA patients (P<0.05). Flow cytometry showed that VEGF significantly inhibited apoptosis of MSCs by blocking S phase cells (P<0.05).@*CONCLUSION@#The activation of VEGF-Notch can restore the proliferation function of MSC in AA patients.


Subject(s)
Anemia, Aplastic , Apoptosis , Bone Marrow , Cell Proliferation , Humans , Mesenchymal Stem Cells , Signal Transduction , Vascular Endothelial Growth Factor A
20.
ABCS health sci ; 43(2): 69-76, 02 ago. 2018. ilus, tab, graf
Article in Portuguese | LILACS | ID: biblio-908967

ABSTRACT

INTRODUÇÃO: O transplante de células-tronco hematopoéticas (TCTH) é a única alternativa para o tratamento de algumas doenças. Entretanto, identifica-se escassez de estudos na população brasileira. OBJETIVO: Caracterizar o perfil dos pacientes submetidos ao TCTH no Hospital de Clínicas da Universidade Federal do Paraná (HC/UFPR), entre 2011 e 2015, com base em variáveis demográficas, diagnóstico, duração da internação, e a taxa de mortalidade na instituição. MÉTODOS: Pesquisa de séries temporais baseada em dados do Sistema de Informações Hospitalares. Avaliou-se a tendência na distribuição das proporções ao longo dos anos por meio do teste de Cochran­Armitage e da regressão binomial negativa. A presença de Autocorrelação seriada foi testada pelo teste de Durbin-Watson. RESULTADOS: De 2011-2015 o Paraná foi responsável por 9,2% dos TCTH realizados no Brasil. O HC/UFPR foi responsável por 46,0% destes procedimentos realizados no Paraná. Não foram observadas variações significativas na distribuição das variáveis sexo (p=0,788) e número de TCTH (p=0,213). 59,5% dos pacientes residiam no PR, 49,4% tinham entre 0 e 17 anos, 79,9% eram brancos, e 63,5% do sexo masculino. O TCTH alogênico foi o mais realizado (88,5%). 58,5% permaneceram internados de 31 a 60 dias (média=37,6 dias). 9,1% foram a óbito. A anemia aplástica adquirida foi a doença base mais frequente (31,9%). CONCLUSÃO: O TCTH é um procedimento de alto custo e complexidade. O estudo e a compreensão dos fatores determinantes para o seu sucesso são de extrema importância para o melhor planejamento, estimativa de risco e elaboração de políticas públicas de saúde.


INTRODUCTION: Hematopoietic stem cell transplantation (HSCT) is the only alternative for the treatment of some diseases. However, there is a shortage of studies in Brazilian population. OBJECTIVE: To identify the profile of patients submitted to HSCT at the Hospital de Clínicas da Universidade Federal do Paraná (HC/UFPR), between 2011 and 2015, based on demographic variables, diagnosis, duration of hospitalization and the mortality ration in the institution. METHODS: Time Series Studies research based on data from the Hospital Information System. The trend in the distribution of proportions over the years was evaluated through Cochran­Armitage test and negative binomial regression. The presence of serial autocorrelation was tested by the Durbin­Watson test. RESULTS: From 2011-2015 Paraná was responsible for 9.2% of HSCT performed in Brazil. HC/UFPR accounted for 46.0% of these procedures performed in Paraná. There were no significant variations in the sex distribution (p=0.788) and number of HSCT (p=0.213). 59.5% of the patients were from PR, 49.4% were between 0 and 17 years old, 79.9% were white, and 63.5% were male. The allogeneic HSCT was the most performed procedure (88.5%). 58.5% were hospitalized from 31 to 60 days (mean=37.6 days). 9.1% died. Acquired aplastic anemia was the most common underlying disease (31.9%). CONCLUSION: HSCT is a procedure of high cost and complexity. The study and the understanding of the determinants of its success are of extreme importance for the best planning, risk estimation and elaboration of public health policies.


Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Adult , Middle Aged , Aged , Time Series Studies , Hematopoietic Stem Cell Transplantation , Anemia, Aplastic/epidemiology , Hospitals, Public , Hospitals, University
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