Comparison between horse and rabbit antithymocyte globulin as first-line treatment for patients with severe aplastic anemia: a single-center retrospective study.

The best antithymocyte globulin preparation for first-line immune suppression in patients with severe aplastic anemia is still not clear. The aim of this study was to compare hematological response and overall survival in patients submitted to horse or rabbit antithymocyte globulin as first-line treatment for severe aplastic anemia. We retrospectively compared 71 consecutive patients with severe aplastic anemia, classified according to the antithymocyte globulin preparation. Analyses included variables related to patients and to immune suppression. Forty two patients (59.1%) received horse and 29 (40.9%) rabbit antithymocyte globulin. Response rates were higher at 6 months in patients submitted to horse in comparison to rabbit antithymocyte globulin (59.5% versus 34.5% respectively, p = 0.05). Median time to response was similar between the two groups (99 versus 88.5 days, respectively, for horse and rabbit antithymocyte globulin; p = 0.98). Overall survival at 2 years was significantly higher in patients submitted to horse in comparison to rabbit antithymocyte globulin (78.4% versus 55.4%, p = 0.03). Post-treatment response was strongly associated with survival at 2 years (97% in responders versus 41.2% in non-responders, p < 0.001). Use of rabbit antithymocyte globulin was an independent predictor of death (odds ratio 2.5; 95% confidence interval 1.03-6.04; p = 0.04). Rabbit antithymocyte globulin was associated with a significant and prolonged lymphopenia in comparison with horse antithymocyte globulin. Our data suggest the superiority of horse over rabbit antithymocyte globulin as first-line treatment for severe aplastic anemia, both regarding hematological response and survival.

High CD8+ lymphocyte dose in the autograft predicts early absolute lymphocyte count recovery after peripheral hematopoietic stem cell transplantation.

Early lymphocyte recovery (ELR) after autologous peripheral hematopoietic stem cell transplantation (ASCT) is an independent predictor for survival in patients with hematological and non-hematological cancers. Sixty-five ASCT for hematological cancers were retrospectively analyzed to identify the factors associated with ELR and to assess the impact of different mobilization regimens on the pre-collection absolute lymphocyte count (ALC). The CD8+ lymphocyte dose in the autograft and the pre-mobilization ALC were independently associated with ELR (P < 0.001 and P = 0.008, respectively). CD8+ lymphocyte doses higher than 0.1 x 10(9)/kg were strongly associated with ELR [P < 0.001, odds ratio 25.22, 95% confidence interval (CI) 4.98-127.69] and this cutoff may be used to predict ELR (P = 0.001, area under the curve 0.75, 95% CI 0.62-0.88). Mobilization with granulocyte colony-stimulating factor (G-CSF) alone, the pre-collection ALC and the number of apheresis sessions were independently associated with the CD8+ lymphocyte dose (P = 0.04, P = 0.001, and P < 0.001, respectively). The number of aphereses was the variable with the strongest correlation to the CD8+ lymphocyte dose (r(s) = 0.68, P < 0.001). Median pre-mobilization ALC was higher than pre-collection ALC in the subgroup of patients without ELR mobilized with chemotherapy followed by G-CSF (1090 vs. 758 lymphocytes/microL; P < 0.001). This reduction was not significant in the subgroup with ELR mobilized with chemotherapy plus G-CSF (1920 vs. 1539/microL, respectively; P = 0.23). These results suggest that the CD8+ lymphocyte dose in the autograft is critical for ELR after ASCT and also demonstrates that mobilization with chemotherapy followed by G-CSF significantly decreases the pre-collection ALC, especially in patients with low pre-mobilization ALC.

Consenso brasileiro sobre atividades esportivas e militares e herança falciforme no Brasil - 2007 / Brazilian consensus of sickle cell trait in sports and army activities

A reunião de consenso brasileiro sobre atividades esportivas e militares e herança falciforme foi realizada no dia 3 de setembro de 2007, no Rio de Janeiro, e reuniu especialistas, representantes das Forças Armadas e de associações de pacientes de doença falciforme. Questões relativas à prática de esporte amador e profissional e do serviço militar foram amplamente discutidas, tendo como base a literatura científica e a experiência de cada um dos participantes. Ao final, algumas recomendações foram assim definidas: 1. O indivíduo portador de traço pode fazer qualquer modalidade de esporte, já que não há dados epidemiológicos consistentes que impeçam a prática de qualquer esporte; 2. Não é necessário fazer triagem para hemoglobinopatias em indivíduos que queiram praticar esportes, quer de natureza amadora ou profissional; 3. Para servir às Forças Armadas não é necessário fazer teste de triagem para hemoglobinopatias, o que equivale dizer que os portadores de traço falciforme podem serviràs Forças Armadas; 4. É fundamental que se esclareça entre os mais diferentes segmentos da sociedade que a heterozigose para a hemoglobina S não confere ao seu portador maior risco que a população geral no que tange às atividades físicas, desde que atendidas as condições básicas de hidratação e de descanso.

The Brazilian consensus meeting concerning sports and military activities and the sickle cell trait was held on September 3rd, 2007 in Rio de Janeiro and brought together experts, and members of the armed forces and sickle cell disease associations. Issues related to the practice of professional and amateur sports and military service were widely discussed based on the scientific literature and the experience of each participant. These were the final recommendations: 1. an individual with sickle cell trait can practice any type of sport because there is no consistent epidemiological data to recommend the contrary; 2. hemoglobin screening is not needed for individuals who want to practice sports, whether as amateur or professional sportspeople; 3. it is not necessary to undergo a screening test for hemoglobin to serve the armed forces, which means that the carriers of the sickle cell trait can serve the armed forces; 4. it must be made clear to the different segments of society that heterozygosis for hemoglobin S does not confer any specific risk to the practice of physical activities, provided the basic conditions of hydration and rest are observed.

Crises dolorosas na doença falciforme / Painful episodes in sickle cell disease

A doença falciforme é a enfermidade genética mais prevalente em todo o mundo. No Brasil, ela ocorre em um a cada 1.200 nascimentos. Por essa alta prevalência, ela representa, em nosso país, um importante problema de saúde pública. Além da hemólise, a vasoclusão é o achado central da doença e responsável pelas crises dolorosas, que correspondem à principal causa de internação nos adultos. Alguns pacientes evoluem com seis ou mais episódios dolorosos por ano. As crises graves e persistentes constituem, nesses pacientes, fator de mau prognóstico e um fator preditivo para morte precoce. Este artigo enfoca as abordagens terapêuticas das crises vasoclusivas nos pacientes de doença falciforme.

Sickle cell disease (SCD) is the most prevalent genetic disease in the world. In Brazil it occurs in one in every 1200 births. This high prevalence makes SCD a very important public health problem in Brazil. Vaso-occlusion and hemolysis are the hallmarks of the disease. Vaso-occlusion results in painful episodes which are the main cause of hospitalization among adults with SCD. Some patients experience episodes as often as 6 times per year. Persistent, severe sickle cell pain is a poor prognostic sign and a predictor for early death. The management of vaso-occlusion episodes is discussed here, as well as the related complications.