Journal of Blood Disorders and Transfusion最新文献

{"title":"Idiosyncratic Agranulocytosis in Elderly Patients","authors":"R. Mourot-Cottet, O. Keller, E. Andrès","doi":"10.4172/2155-9864.1000351","DOIUrl":"https://doi.org/10.4172/2155-9864.1000351","url":null,"abstract":"Sixty-one patients were identified. One patient presented 2 episodes of IDIA (with 2 different drugs). All patients were Caucasian. The mean and median ages were 84.9 and 82 years (range, 75-95), respectively. The sex-ratio female/male was 2.4. Seventy-four percent of the patients (n=45) presented an underlying disease. Main families of causative drugs were: antibiotics (n=25, 43.8%), especially ß-lactams (n=16) and cotrimoxazole (n=9); antithyroid drugs (n=9, 15.8%); neuroleptic and anti-epileptic agents (n=7, 12.3%); and antiaggregative platelet agents (n=6, 10.5%), especially ticlopidine (n=5). There was only 1 case of self-medication. Discovery circumstances were (n=60): decrease in neutrophil blood counts (n=29, 48.3%); isolated fever (n=22, 36.7%); documented infections (n=8, 13.3%), as acute tonsillitis or pneumonia, with one case of septic shock. Clinical presentations during hospitalization were (n=58): isolated fever (fever of unknown origin) (n=16, 27.6%); documented pneumonia (n=12, 20.7%); septicemia (n=9, 15.5%); septic shock (n=5, 8.6%). The remaining symptomatic patients presented documented infections (n=14, 24.1%), as: acute pyelonephritis (n=4); sore throats and acute tonsillitis (n=4); cutaneous infections (n=1); cholecytitis (n=1); colitis (n=1); infectious spondylitis (n=1); endocarditis (n=1); and fever with deep venous thrombosis (n=1). Two patients (3.4%) remained asymptomatic during the hospitalization. During the hospitalization, 15 patients (25.9%) presented features of severe sepsis, septic shock and/or systemic inflammatory response syndrome (SIRS). Bacteriological documentation (n=54) was obtained in 23 cases (42.6%). At the time of discovery, the mean and median neutrophil counts were 1.62 and 1.5 × 109/L (range, 0.26-6.3). At nadir of the neutrophil decrease, mean and median neutrophil counts were 0.15 and 0.08 × 109/L (range, 0-0.4). Fifty-four percent of patients (n=33) had neutrophil levels of less than 0.1 × 109/L.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"65 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2016-05-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133282261","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Strategic Business Planning in Key to Effective Diagnosis and Management of Blood Disorders in Developing Countries","authors":"O. Erhabor, Y. Abdulrahaman, Egenti Bn, H. Buhari, C. Fredricks, T. Adias, B. Echonwere, Okara Gc","doi":"10.4172/2155-9864.1000350","DOIUrl":"https://doi.org/10.4172/2155-9864.1000350","url":null,"abstract":"","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"48 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2016-05-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132209420","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Recovery from Cidofovir-Resistent BK Virus-Associated Hemmorhagic Cystitis Post Cord Blood Transplant Following Rapid Taper of Immunosuppression","authors":"L. Xiaofan, H. Fu, Li Nainong, Ji, A. Hu, Yuanzhong Chen","doi":"10.4172/2155-9864.1000348","DOIUrl":"https://doi.org/10.4172/2155-9864.1000348","url":null,"abstract":"Hemorrhagic cystitis (HC) usually happens 2-4 weeks after Hematopoietic Stem Cell Transplantation (HSCT). BK virus associated HC is a severe complication after HSCT and usually sensitive to cidofovir treatment. Here we report a late onset BK virus-associated HC after a cord blood transplantation (HLA 5/6) followed by a failure treatment of cidofovir in an acute lymphoblastic leukemia patient. The patient was conditioned with an intensified conditioning regimen containing fludarabine, cytosine arabinoside, busulfan, cyclophosphamide and rabbit antithymocyte globulin (FABuCy+ATG). The late development of HC with the symptom of hematuria occurred 22 days after HSCT and was associated with BK virus. In two weeks, the hematuria progressed with the failure of hydration, alkalizing diuresis and reduction of immunosuppressant treatment. Surprisingly, treatment of cidofovir only transient lower BK virus copies and was proved to be a failure. Thereafter, early immunosuppressant withdrawal was applied followed by thymosin α1 injections to enhance immunity. Although the BKV was still high, HC recovered with mild and controllable GVHD. One year after HSCT, the patient remain well without HC as well as GVHD.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"12 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2016-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125310456","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Innovative immunotherapy for prevention and treatment of relapse following conventional chemotherapy or stem cell transplantation","authors":"S. Slavin","doi":"10.4172/2155-9864.C1.012","DOIUrl":"https://doi.org/10.4172/2155-9864.C1.012","url":null,"abstract":"","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"177 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2016-04-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123416327","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Congenital Prothrombin Deficiency: A Rare Cause of Puberty Menorrhagia","authors":"Swaramya Ch, rasekaran, H. Sagili, P. Dasari","doi":"10.4172/2155-9864.1000346","DOIUrl":"https://doi.org/10.4172/2155-9864.1000346","url":null,"abstract":"During the transitioning through puberty, adolescents present with varied gynaecological issues, among which puberty menorrhagia is a significant complaint. The most common underlying cause of puberty menorrhagia is anovulation, other causes being endocrine dysfunction, PCOS and bleeding disorders. Congenital prothrombin deficiency is an extremely rare inherited coagulopathy, affecting one in two million of general population. Depending on the severity of deficiency they present with myriad bleeding tendencies, including epstaxis, soft tissue bleeds, GI hemorrhage, intracranial hemorrhage, menorrhagia, excessive post traumatic and post-surgical bleeding. Here, we present a very rare case of congenital prothrombin deficiency presenting primarily with puberty menorrhagia.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"163 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2016-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127524938","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of Trace Elements in Adult Sickle Cell Anaemia Patients in Zaria, North Western Nigeria","authors":"N. Garba, Ifeanyichukwu Om, Amilo Gi, I. Audu","doi":"10.4172/2155-9864.1000347","DOIUrl":"https://doi.org/10.4172/2155-9864.1000347","url":null,"abstract":"Sickle cell anemia is a genetic disorder that is caused due to the inheritance of a mutant gene that encodes haemoglobin S (HbS). The amino acid glutamic acid is replaced with valine in the sixth position of b-globin chain. Protection of red cell membrane from free radical mediated oxidative stress is crucial to the management of SCD. Trace elements such as Copper, Zinc and Magnesium are of great benefit towards relieve of oxidative stress associated with red blood cell membranes. This study was carried out to evaluate some trace elements in adult sickle cell anaemia patientsattending sickle cell clinic, ABUTH-Zaria. Ethical clearance was obtained from ethics committee of Ahmadu Bello University Hospital Zaria, questionnaires were administered and informed consent was obtained from patients or their parents. One hundred and one (101) subjects aged 18 to 46 years participated in this study and these participants were divided into thirty five (35) confirmed sickle cell anaemia subjects in stable state (SS), thirty five (35) confirmed sickle cell anaemia subjects with history of vaso-occlusive crises in the last three months and (31) apparently healthy subjects (Hb AA) as control subjects (C). Haemoglobin electrophoresis was done using cellulose acetate method and serum copper, zinc and magnesium was analysed using Atomic Absorption Spectrophotometer (AAS) method. Serum copper, zinc and magnesium mean levels were significantly lower (P = 0.00) in SCA (SS and VOC) groups when compared with control group. No significance difference was observed in the mean levels of copper, zinc and magnesium in SS group when compared with VOC group (P = 0.36, P = 0.89 and P = 0.85) respectively. The mean levels of trace elements were significantly lower in SCA groups than the control group. Evaluation of trace elements is suggested in the management of sickle cell anaemia.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"56 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2016-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"128537449","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Health Related Quality of Life of Patients with Sickle Cell Disease","authors":"M. Matos, Cristiane Dias Malheiros, Simone Souza da Rocha Matos","doi":"10.4172/2155-9864.1000345","DOIUrl":"https://doi.org/10.4172/2155-9864.1000345","url":null,"abstract":"Objective: The aim of the present study was to evaluate the health-related quality of life in pediatric patients with Sickle Cell Disease. \u0000Methods: An analytical cross-sectional study was performed. Pediatric patients suffering from Sickle Cell Disease below the age of 21 were compared with asymptomatic pediatric individuals. Clinical and demographic data were collected along with the Pediatric Quality of Life Inventory (PedsQL 4.0). \u0000Results: The sample was composed of 68 children and adolescents in the \"Sickle cell group\" and 44 in the \"Comparison group\". Children and adolescent with SCD had lower scores of health related quality of life compared with healthy subjects in the domains physical (68.3 versus 88.8), social (71.1 versus 90.7), and school functioning (60.2 versus 78.7). The domain emotional functioning was not significantly different (62.1 versus 66.5). \u0000Conclusion: The results of the present study demonstrated that children and adolescents with SCD had lower scores of quality of life in the domains activities, social and school functioning when compared to healthy pediatric individuals.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"38 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2016-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133113251","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Transfusion of ABO-Incompatible HLA-Matched Platelets as Support for Patients with Acute Myeloid Leukaemia Undergoing Chemotherapy","authors":"S. Yamasaki, J. Fujimoto, K. Kohno, M. Kadowaki, Sachiko Matsunaga, K. Takase, S. Okamura","doi":"10.4172/2155-9864.1000344","DOIUrl":"https://doi.org/10.4172/2155-9864.1000344","url":null,"abstract":"Background: ABO-incompatible human leukocyte antigen (HLA)-matched platelets are used to manage thrombocytopenic patients with anti-HLA-A and/or anti-HLA-B (HLA-A/B) antibodies when ABO-identical donors are not available. This study assessed the effectiveness of ABO-incompatible HLA-matched platelets in an unselected group of patients with acute myeloid leukaemia (AML) undergoing cytotoxic chemotherapy. \u0000Material and Methods: The study cohort consisted of 12 AML patients undergoing cytotoxic chemotherapy and administered HLA-matched single donor platelet transfusions. Patients positive for anti-HLA-A/B antibodies were defined as candidates for HLA-matched platelet transfusion. The effectiveness of platelet transfusions was determined by measuring corrected count increments (CCIs). \u0000Results: The 12 patients received a total of 128 HLA-matched platelet transfusions. The median CCIs 1 hour after ABO-minor and -major incompatible HLA-matched transfusions were 11.4 (range: 3.2-24.9) and 12.4 (range: 3-37), respectively. There were no significant differences in 1- and 24-hour CCIs among ABO-identical and ABOminor and major incompatible transfusions. \u0000Discussion: ABO-incompatible HLA-matched platelets are effective in supporting thrombocytopenic AML patient’s positive for anti-HLA-A/B antibodies and undergoing cytotoxic chemotherapy.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"54 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2016-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125675192","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hematologic Disturbances and Neurosurgery: Why Dont We Consider Them?","authors":"M. Luongo","doi":"10.4172/2155-9864.1000343","DOIUrl":"https://doi.org/10.4172/2155-9864.1000343","url":null,"abstract":"There are some conditions in neurosurgical practice that could be as common as insidious, especially if presented by people without risk factors. This could be the case of Chronic Subdural Hematoma (CSDH), considered a disease of the elderly, with a low morbidity among young people. The well recognized hypothesis about the genesis of CSDH (head injury, coagulopathies, hemostatic disorders, antiaggregant therapy etc.) could not always explain its developing mechanism among young people. Because of the unspecific symptoms and insidious onset in juveniles and young adults, CSDH could be neglected even undertreated in that population group but many reports suggest some other predisposing factors may exist.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"12 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2016-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121738865","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Anti-CD19 Chimeric Antigen Receptor-Modified T Cells for Multiple Myeloma","authors":"Satadal Barik","doi":"10.4172/2155-9864.1000342","DOIUrl":"https://doi.org/10.4172/2155-9864.1000342","url":null,"abstract":"Multiple myeloma is a disease formed by malignant plasma cells that accumulate in the bone marrow. The lifetime risk of multiple myeloma in the U.S. is 1/140, making it the second most common hematologic malignancy and it was expected that 22,350 new cases to be diagnosed in the US in 2013 [1]. In the past decade, we have witnessed dramatic changes in the treatment of multiple myeloma. Proteasome inhibitors such as bortezomib and carfilzomib target the ubiquitin pathway, resulting in cytotoxic injury due to disruption of protein degradation in myeloma cells. When compared with melphalan–prednisone–lenalidomide (MPR), combine therapy with high-dose melphalan and stem-cell transplantation significantly prolonged progression-free survival among patients with multiple myeloma who were in the age group of 65 years or younger [2]. Discovery of anti-tumor efficacy of the graft vs. myeloma response, such as CAR T cell therapy seems likely to herald the beginning of a revolution in the treatment of multiple myeloma. Several observations have fostered optimism that more specific immunotherapeutic approach such as chimeric antigen receptors (CARs) modified autologous T cells might exhibit more potent anti-myeloma activity with less toxicity. Currently all ongoing CAR studies employ firstgeneration anti-CD19 CAR and a second-generation version, usually with CD28. In pre-clinical studies, anti-myeloma second-generation CARs are developed to target the Lewis Y antigen (Ley), B Cell Maturation Antigen (BCMA), cell surface glycoprotein CS1 and CD38. Ley is overexpressed in many epithelial malignancies. In a recent study, multiple myeloma cell lines were injected into a immunocompromised mice followed by the injection of anti-Ley CAR T cells that significantly exhibited prolonged survival and delayed development of symptomatic plasmacytomas [3]. The National Cancer Institute (NCI) has recently identified BCMA which is expressed on most multiple myeloma cells. Anti-BCMA CAR is a second-generation CD28-based CAR that showed quite favourable efficacy and toxicity profiles against multiple myeloma [4]. CS1 is over-expressed in multiple myeloma cells. The CD28-based anti-CS1 CAR-modified natural killer (NK) cells revealed cytotoxicity against multiple myeloma cell lines and prolonged survival in patients with multiple myeloma [5]. CD38 is a transmembrane glycoprotein and a second-generation 4-1BB-based anti-CD38 CAR was proved to be effective against multiple myeloma cell lines [6]. In a phase I/II study, anti-CD38 antibody daratumumab showed a remarkable efficacy for the treatment of patients with multiple myeloma [7]. The most clinical experience so far has been with anti-CD19 CARs that have been utilized in several phase I trials targeting B cell malignancies. Genetically engineered CARs that couple with an anti-CD19 single chain Fv domain to intracellular T-cell signalling domains of the T-cell receptor transmit cytotoxic T lymphocytes to antigen expressing cells.","PeriodicalId":182392,"journal":{"name":"Journal of Blood Disorders and Transfusion","volume":"197 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2016-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133411697","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}