RESUMO
Aplastic anemia (AA) is a rare and serious disorder of hematopoietic stem cells (HSCs) that results in the loss of blood cells due to the failure of the bone marrow (BM). Although BM transplantation is used to treat AA, its use is limited by donor availability. In this sense, mesenchymal stem cells (MSCs) can offer a novel therapeutic approach for AA. This is because the MSCs contribute to the hematopoietic niche organization through their repopulating. In our study, we used the human immature dental pulp stem cell (hIDPSC), an MSC-like cell, to explore an alternative therapeutic approach for AA. For this, isogenic C57BL/6 mice were exposed to total body irradiation (TBI) to induce the AA. After 48 h of TBI, the mice were intraperitoneally treated with hIDPSC. The immunohistochemistry analyses confirmed that the hIDPSCs migrated and grafted in the mouse bone marrow (BM) and spleen, providing rapid support to hematopoiesis recovery compared to the group exposed to radiation, but not to those treated with the cells as well as the hematological parameters. Six months after the last hIDPSC transplantation, the BM showed long-term stable hematopoiesis. Our data highlight the therapeutic plasticity and hematoprotective role of hIDPSC for AA and potentially for other hematopoietic failures.
Assuntos
Anemia Aplástica , Células-Tronco Mesenquimais , Anemia Aplástica/etiologia , Anemia Aplástica/terapia , Animais , Polpa Dentária , Hematopoese , Humanos , Camundongos , Camundongos Endogâmicos C57BLRESUMO
Introduction: Pregnancy-associated aplastic anemia (pAA) occurs when aplastic anemia (AA) is diagnosed for the first time during pregnancy and it is a rare but serious condition leading to severe maternal and fetal complications. It is unknown whether pregnancy triggers bone marrow failure or if this state is unrelated to the pathogenesis of pAA.Areas covered: In this review, three new cases of pAA are presented and its controversial etiologic relationship with pregnancy, its atypical presentation, and management are also discussed. Furthermore, a literature review of pAA cases between 1975 and 2020 was performed in PubMed, accessed via the National Library of Medicine PubMed interface. Keywords included 'aplastic anemia' AND 'pregnancy'. We found 54 cases reported in the literature with a clear diagnosis of pAA.Expert opinion: The diagnosis of pAA is challenging since pregnancy is associated with physiologic hematological changes in the complete blood count which can mask the disease. Meticulous monitoring and adequate support therapy given by a trained multidisciplinary team have the potential to improve outcomes for women and their neonates. All women should receive frequent assessments to optimize their care during pregnancy and after delivery, definitive treatment should be offered.
Assuntos
Anemia Aplástica , Pancitopenia , Anemia Aplástica/diagnóstico , Anemia Aplástica/etiologia , Anemia Aplástica/terapia , Contagem de Células Sanguíneas , Feminino , Humanos , Recém-Nascido , Gravidez , Estados UnidosRESUMO
OBJECTIVES: To assess the prognostic role of hepatitis in pediatric patients with aplastic anemia and the incidence of hepatitis B among patients with hepatitis-associated aplastic anemia in an area with a previously high prevalence of hepatitis B after nationwide hepatitis B vaccination for 30 years. STUDY DESIGN: Pediatric patients (n = 78) with aplastic anemia were enrolled in this study, including 9 with hepatitis-associated aplastic anemia. We collected the clinical characteristics, etiologies of the aplastic anemia, hepatitis B virus serology and serum hepatitis B viral load, response to the treatments, and survival outcome from the participants. We applied univariate and multivariate Cox regression analysis to evaluate the correlations between clinical features and survival outcome. Survival analysis was done using Cox regression model and Kaplan-Meier curves. RESULTS: Patients with hepatitis-associated aplastic anemia were related to significantly worse survival prognosis when compared with patients with non-hepatitis-associated aplastic anemia, and hepatitis-associated aplastic anemia was the only independent prognostic factor to predict a poor survival outcome in our patients with aplastic anemia by multivariable analysis. In none of the total 78 patients was aplastic anemia related to hepatitis B virus infection. CONCLUSIONS: Patients with hepatitis-associated aplastic anemia had a significantly worse prognosis when compared with patients whose aplastic anemia was not hepatitis-associated. This study demonstrates the potential benefit of hepatitis B vaccination in decreasing the incidence of hepatitis-associated aplastic anemia in children.
Assuntos
Anemia Aplástica/virologia , Hepatite B/complicações , Adolescente , Anemia Aplástica/sangue , Anemia Aplástica/etiologia , Anemia Aplástica/mortalidade , Estudos de Casos e Controles , Criança , Pré-Escolar , Hepatite B/sangue , Vírus da Hepatite B/isolamento & purificação , Humanos , Imunossupressores/uso terapêutico , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Modelos de Riscos Proporcionais , Índice de Gravidade de Doença , TaiwanRESUMO
In spite of significant strides in the treatment of sickle cell disease (SCD), SCD crises are still responsible for high morbidity and early mortality. While most patients initially seek care in the acute setting for a seemingly uncomplicated pain episode (pain crisis or vaso-occlusive crisis), this initial event is the primary risk factor for potentially life-threatening complications. The pathophysiological basis of these illnesses is end-organ ischemia and infarction combined with the downstream effects of hemolysis that results from red blood cell sickling. These pathological changes can occur acutely and lead to a dramatic clinical presentation, but are frequently superimposed over a milieu of chronic vasculopathy, immune dysregulation, and decreased functional reserve. In the lungs, acute chest syndrome is a particularly ominous lung injury syndrome with a complex pathogenesis and potentially devastating sequelae, but all organ systems can be affected. It is, therefore, critical to understand the SCD patients' susceptibility to acute complications and their risk factors so that they can be recognized promptly and managed effectively. Blood transfusions remain the mainstay of therapy for all severe acute crises. Recommendations and indications for the safest and most efficient implementation of transfusion strategies in the critical care setting are therefore presented and discussed, together with their pitfalls and potential future therapeutic alternatives. In particular, the importance of extended phenotypic red blood cell matching cannot be overemphasized, due to the high prevalence of severe complications from red cell alloimmunization in SCD.
Assuntos
Síndrome Torácica Aguda/terapia , Anemia Aplástica/terapia , Anemia Falciforme/terapia , Antibacterianos/uso terapêutico , Insuficiência de Múltiplos Órgãos/terapia , Oxigenoterapia , Púrpura Trombocitopênica Trombótica/terapia , Acidente Vascular Cerebral/terapia , Síndrome Torácica Aguda/etiologia , Síndrome Torácica Aguda/fisiopatologia , Anemia Aplástica/etiologia , Anemia Aplástica/fisiopatologia , Anemia Falciforme/complicações , Anemia Falciforme/fisiopatologia , Tipagem e Reações Cruzadas Sanguíneas/métodos , Progressão da Doença , Transfusão de Eritrócitos/métodos , Transfusão Total/métodos , Humanos , Insuficiência de Múltiplos Órgãos/etiologia , Insuficiência de Múltiplos Órgãos/fisiopatologia , Dor/etiologia , Manejo da Dor , Síndrome da Leucoencefalopatia Posterior , Púrpura Trombocitopênica Trombótica/etiologia , Púrpura Trombocitopênica Trombótica/fisiopatologia , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/fisiopatologiaAssuntos
Anemia Aplástica , Ciclofosfamida/administração & dosagem , Transplante de Células-Tronco Hematopoéticas , Hemoglobinúria Paroxística , Adulto , Aloenxertos , Anemia Aplástica/etiologia , Anemia Aplástica/terapia , Hemoglobinúria Paroxística/complicações , Hemoglobinúria Paroxística/terapia , Humanos , MasculinoAssuntos
Anemia Aplástica/etiologia , Medula Óssea/patologia , Hemoglobinúria Paroxística/etiologia , Hepatite Viral Humana/complicações , Anemia Aplástica/patologia , Anemia Aplástica/cirurgia , Exame de Medula Óssea , Transplante de Medula Óssea , Feminino , Hemoglobinúria Paroxística/patologia , Hemoglobinúria Paroxística/cirurgia , Humanos , Pancitopenia/etiologia , Pancitopenia/cirurgia , Adulto JovemRESUMO
Aplastic anemia following viral hepatitis is a condition well recognized in the medical literature. Although hepatitis-associated aplastic anemia is an uncommon syndrome, there are several reports in the literature describing such cases. In these reports, aplastic anemia generally occurs following a viral infection, including parvovirus B19, but may also be idiopathic. The etiology of both the hepatic injury and the bone marrow failure is speculated to be immune-mediated. We report a patient who suffered acute idiopathic hepatitis and severe pancytopenia fourteen years after a similar episode in childhood. This is only the second case report of acute hepatitis in association with bone marrow failure and aplastic anemia in childhood with sudden recurrence many years later in adulthood.
Assuntos
Anemia Aplástica/diagnóstico , Anemia Aplástica/etiologia , Hepatite/complicações , Hepatite/diagnóstico , Pancitopenia/diagnóstico , Pancitopenia/etiologia , Adulto , Anemia Aplástica/tratamento farmacológico , Soro Antilinfocitário/uso terapêutico , Biópsia , Ciclosporina/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Fígado/patologia , Masculino , Pancitopenia/tratamento farmacológico , Recidiva , Fatores de Risco , Fatores de Tempo , Resultado do TratamentoRESUMO
UNLABELLED: Background Associations between aplastic anemia and numerous drugs, pesticides and chemicals have been reported. However, at least 50% of the etiology of aplastic anemia remains unexplained. DESIGN AND METHODS: This was a case-control, multicenter, multinational study, designed to identify risk factors for agranulocytosis and aplastic anemia. The cases were patients with diagnosis of aplastic anemia confirmed through biopsy or bone marrow aspiration, selected through an active search of clinical laboratories, hematology clinics and medical records. The controls did not have either aplastic anemia or chronic diseases. A total of 224 patients with aplastic anemia were included in the study, each case was paired with four controls, according to sex, age group, and hospital where the case was first seen. Information was collected on demographic data, medical history, laboratory tests, medications, and other potential risk factors prior to diagnosis. RESULTS: The incidence of aplastic anemia was 1.6 cases per million per year. Higher rates of benzene exposure (>/=30 exposures per year) were associated with a greater risk of aplastic anemia (odds ratio, OR: 4.2; 95% confidence interval, CI: 1.82-9.82). Individuals exposed to chloramphenicol in the previous year had an adjusted OR for aplastic anemia of 8.7 (CI: 0.87-87.93) and those exposed to azithromycin had an adjusted OR of 11.02 (CI 1.14-108.02). Conclusions The incidence of aplastic anemia in Latin America countries is low. Although the research study centers had a high coverage of health services, the underreporting of cases of aplastic anemia in selected regions can be discussed. Frequent exposure to benzene-based products increases the risk for aplastic anemia. Few associations with specific drugs were found, and it is likely that some of these were due to chance alone.
Assuntos
Agranulocitose/epidemiologia , Anemia Aplástica/epidemiologia , Adolescente , Adulto , Agranulocitose/etiologia , Agranulocitose/patologia , Anemia Aplástica/etiologia , Anemia Aplástica/patologia , Derivados de Benzeno/toxicidade , Medula Óssea , Brasil/epidemiologia , Estudos de Casos e Controles , Criança , Pré-Escolar , Exposição Ambiental/efeitos adversos , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Fatores de RiscoRESUMO
INTRODUCTION: The aim of the study is to report results of erythropoietin treatment for late hyporegenerative anemia in the hemolytic disease of the newborn (HDN). Reports previously published concern only a few cases, with controversial results. METHODS: Case series report concerning 50 neonates with HDN due to Rh, ABO or KpA antigens, aged more than 7 days. Erythropoietin treatment started when hematocrit dropped to levels requiring transfusion, with an inappropriate reticulocyte response (Reticulocyte Production Index <1). RESULTS: At start of treatment mean age was 24.3 +/- 12.0 days (range 8-65 days), hematocrit 24.1 +/- 2.8% (range 18-30%), and Reticulocyte Production Index 0.34 +/- 0.25 (range 0.05-0.98). Hematocrit and Reticulocyte Production Index showed significant increases after 7 and 14 days of treatment (p <0.001). No difference was observed either between infants with Rh-HDN and ABO-HDN or between Rh-HDN patients with or without intrauterine transfusions. Seven infants (14%) required one packed RBC transfusion during erythropoietin therapy, 2 of them within 72 hours from starting treatment. The percentage of transfused infants showed no difference either between ABO-HDN and Rh-HDN or between Rh-HDN with and without intrauterine transfusions. Moderate, short-lasting neutropenia, not associated to infections, was observed in 11 patients. No other adverse effect was observed. CONCLUSIONS: The administration of erythropoietin appears to be a safe and useful therapy. Its efficacy should be confirmed by randomized studies.
Assuntos
Anemia Aplástica/tratamento farmacológico , Anemia Aplástica/etiologia , Eritroblastose Fetal , Eritropoetina/uso terapêutico , Humanos , Lactente , Recém-Nascido , Proteínas RecombinantesRESUMO
Aplastic anemia is a medullary insufficiency secondary to the complete or partial disappearance of hematopoietic tissue without abnormal cellular proliferation. This is a rare complication of infections, such as dengue hemorrhagic fever. A 15-year-old girl was admitted with anemia, bleeding from the gums, petechiae and fever. Laboratory tests at the time of admission showed: Hemoglobin 4.8 g/dl; Hematocrit 13.4%; white blood count 2240/microl and platelets 11,500/microl. Dengue virus IgM antibodies were found. A bone marrow aspirate and biopsy showed severe aplastic anemia. Treatment with intravenous immunoglobulin and methylprednisolone was started. The patient had a favorable outcome. She was then given long-term treatment with cyclosporin. She is now in remission, without any symptoms. Dengue can induce aplastic anemia through direct bone marrow invasion. This is a rare complication which must be identified early. Immunosuppressive therapy can induce complete remission.