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PURPOSE: Primary immune thrombocytopenic purpura (ITP), caused by immune system dysfunction, is recognized as the leading cause of thrombocytopenia in pediatric population. Nonetheless, inadequate studies have been performed on bone marrow immunophenotyping of children with ITP. In this study, we aimed to investigate the immunophenotype of bone marrow lymphocytes in these children. PATIENTS AND METHODS: Between 2008 and 2012, 35 children with ITP and 26 age and sex matched healthy controls were recruited. All participants underwent bone marrow aspiration. Appropriate B-cell, T-cell, and myeloid lineage monoclonal antibodies were employed to determine the immunophenotype of these patients. RESULTS: CD10, CD19, and CD20, all indicative of premature B-cell markers, were significantly greater in children with ITP. CD22, mainly expressed on mature B cells was slightly, but not significantly reduced in the patients' group (P = .42). On the other hand, T cell markers including CD2, CD3, CD5, and CD7 were underexpressed. CD33, a specific marker for myeloid lineage, was underexpressed in the patients' group (5.6 ± 4.7 vs. 12.9 ± 7.3, P < .001). Noteworthy, the immunophenotype did not significantly differ between acute and persistent cases. CONCLUSION: Overall, a phenotype characterized by increased pre-B-cell markers along with decreased T cell immunophenotypic markers was observed in bone marrow lymphocytes of children with ITP in the present study. Further larger scale studies are recommended to confirm our findings, as precise mapping of the immunophenotype of lymphocytes in these patients would pave the road to improved diagnosis and treatment.
Assuntos
Linfócitos B/imunologia , Células Mieloides/imunologia , Púrpura Trombocitopênica Idiopática/imunologia , Púrpura Trombocitopênica Idiopática/patologia , Linfócitos T/imunologia , Adolescente , Linfócitos B/patologia , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Citometria de Fluxo , Humanos , Imunofenotipagem , Lactente , Subpopulações de Linfócitos/imunologia , Subpopulações de Linfócitos/patologia , Masculino , Células Mieloides/patologia , Linfócitos T/patologiaRESUMO
INTRODUCTION: Herein, the results of a prospective study evaluating the efficacy and safety of treatment with deferasirox are studied in iron-overloaded patients with ß-thalassemia major during an 18-month trial. METHODS: Thirty patients who were previously chelated with deferoxamine with/without deferiprone, and fulfilled the inclusion criteria were recruited. Patients received an initial dose of 10-30 mg/kg/day. Liver and cardiac MRI T2* were evaluated before and after the trial. In addition, serum ferritin level was assessed every 3 months. Primary endpoint was regarded as significant improvement in the severity of liver and cardiac iron overload in severe and moderate cases, in addition to improvement or maintenance of the grade of severity in patients with mild iron overload or normal iron accumulation. Therapy was considered effective if primary endpoint was met in >50%. RESULTS: Liver MRI values improved significantly (P = .002), achieving a 73.33% success rate. A successful outcome regarding myocardial iron overload was observed in 80%. Finally, an overall of 66.66% of patients met the success criteria. Secondary endpoint, regarded as safety and tolerability was reached by 93.33%. The most common adverse events were skin rash and gastrointestinal disturbance. A dose between 30 and 40 mg/kg/day, tailored to each patient was considered the optimal dose. CONCLUSION: Deferasirox proved as an efficient and safe chelating agent in our patients, specifically in mild to moderate iron overloaded patients.
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Benzoatos/uso terapêutico , Terapia por Quelação , Quelantes de Ferro/uso terapêutico , Sobrecarga de Ferro/tratamento farmacológico , Triazóis/uso terapêutico , Talassemia beta/complicações , Adolescente , Adulto , Benzoatos/administração & dosagem , Benzoatos/efeitos adversos , Terapia por Quelação/efeitos adversos , Criança , Pré-Escolar , Deferasirox , Toxidermias/etiologia , Feminino , Ferritinas/sangue , Gastroenteropatias/induzido quimicamente , Humanos , Irã (Geográfico)/epidemiologia , Ferro/análise , Quelantes de Ferro/administração & dosagem , Quelantes de Ferro/efeitos adversos , Sobrecarga de Ferro/etiologia , Sobrecarga de Ferro/patologia , Fígado/química , Fígado/patologia , Imageamento por Ressonância Magnética , Masculino , Miocárdio/química , Miocárdio/patologia , Estudos Prospectivos , Volume Sistólico , Reação Transfusional , Resultado do Tratamento , Triazóis/administração & dosagem , Triazóis/efeitos adversos , Adulto Jovem , Talassemia beta/epidemiologia , Talassemia beta/terapiaRESUMO
An important complication of chemotherapy is thromboembolic events that can occur during treatment course. In this way, Warfarin can be used as an efficient prophylactic agent to prevent these complications. Although bleeding is a common adverse effect of Warfarin, eosinophilia is a rare side effect of this drug. We have reported a 5-year-old boy with Burkitt lymphoma who underwent chemotherapy. In the course of chemotherapy, because of thrombosis of the left jugular vein, we initiated Warfarin as a prophylactic drug for TEE secondary to chemotherapy. Following Warfarin initiation, eosinophilia appeared and subsequent to cessation of drug, eosinophilia disappeared. This case is presented to point out physicians to consider eosinophilia as a rare adverse-effect of Warfarin and monitor blood cell differentiation regularly during the course of treatment with this drug.
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Betacoronavirus , Infecções por Coronavirus/complicações , Deficiência do Fator X/complicações , Hemorragia Gastrointestinal/etiologia , Pneumonia Viral/complicações , Betacoronavirus/isolamento & purificação , COVID-19 , Teste para COVID-19 , Cesárea , Técnicas de Laboratório Clínico , Consanguinidade , Infecções por Coronavirus/sangue , Infecções por Coronavirus/congênito , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/transmissão , Deficiência do Fator X/congênito , Feminino , Hemorragia Gastrointestinal/terapia , Hemostáticos/uso terapêutico , Humanos , Recém-Nascido , Transmissão Vertical de Doenças Infecciosas , Pandemias , Plasma , Pneumonia Viral/sangue , Pneumonia Viral/congênito , Pneumonia Viral/transmissão , Gravidez , Complicações Infecciosas na Gravidez/virologia , Reação em Cadeia da Polimerase Via Transcriptase Reversa , SARS-CoV-2 , Ácido Tranexâmico/uso terapêutico , Adulto JovemRESUMO
Kawasaki disease (KD) is a systemic vasculitis of unknown etiology and a leading cause of acquired heart disease. It is assumed that there is an activation of the immune system by an infectious trigger in a genetically susceptible host. Neuroblastoma is the most common extracranial solid tumor in young children. It mainly originates from primordial neural crest cells that generate the adrenal medulla and sympathetic ganglia. A diagnosis of concurrent KD and neuroblastoma in a living child has been made in only one previous report. We report the second case and review the literature.
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The primary intrarenal neuroblastoma (IRNB) is a rare condition. Intrarenal neuroblastoma typically results from direct renal invasion from an adrenal neuroblastoma, but true intrarenal neuroblastoma originates either sequestered adrenal rests during the fetal life or intrarenal sympathetic ganglia. Clinical, radiological, and pathological correlation is very essential for diagnosis and appropriate management of this type of unusual cases. The distinction of this rare tumor from Wilms' tumor is an important challenge since both tumors have major differences in prognostic and therapeutic response. We present a 3-year-old boy of primary intrarenal neuroblastoma with extensive abdominal and mediastinal mass, persistent hypertension, and disseminated intravascular coagulation (DIC).
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BACKGROUND: Infection is now the most common cause of morbidity in Systemic Lupus Erythematosus (SLE). There is lack of information regarding the specific antibody formation in response to vaccines in young SLE patients. OBJECTIVE: To determine the efficacy of anti-tetanus antibody response in young patients with SLE. METHODS: Forty SLE patients with mean age of 14.1 years (range: 7-21) and 60 age and sex matched normal controls were enrolled in this study over a period of one year. Diagnosis was made according to the ACR criteria and disease activity was determined based on SLE Disease Activity Index (SLEDAI). All patients and controls had received the complete schedule of tetanus vaccinations consisting of three primary doses and two boosters by the age of six. Serum immunoglobulins and anti-tetanus antibody titers were determined by Nephelometry and ELISA. Anti-tetanus antibody levels greater than 0.1 IU/ml have been suggested as protective. RESULTS: In all of the patients and controls anti-tetanus antibody titer was > 0.1 IU/ml. IgG, IgA, and IgM levels were in the normal range for their age. Mean disease activity score was 4.9 (range: 0-16). There was no association between SLEDAI score and anti-tetanus antibody response. CONCLUSION: School age onset and immunosuppressive therapy does not seem to interfere with development of consistent immunity to tetanus vaccine in young SLE patients.