Monique Loureiro

Hyperhemolysis syndrome in patients with sickle cell anemia: report of three cases

Sickle cell anemia (SCA) is characterized by chronic hemolytic anemia. Worsening of anemia after red blood cell (RBC) transfusion has been reported and is often referred to as hyperhemolysis syndrome (HS). HS is a severe transfusion reaction characterized by destruction of both donor and host RBCs.

Acute clinical events in patients with sickle cell disease: epidemiology and treatment

A doenca falciforme e uma doenca hereditaria, de alta prevalencia na populacao negra, que leva a multiplas internacoes hospitalares. Nosso objetivo foi descrever e analisar o curso clinico de pacientes com doenca falciforme hospitalizados.Realizou-se estudo transversal de 78 pacientes submetidos a 230 internacoes hospitalares devido a complicacoes agudas da doenca falciforme, de 2000 a 2004, em um hospital universitario no Rio de Janeiro-RJ, Brasil. Os desfechos estudados foram tempo de permanencia hospitalar e obito. As principais co-variaveis foram idade, sexo, presenca de insuficiencia renal cronica, causas de hospitalizacao e uso de medicamentos. Proporcoes foram comparadas utilizando-se o teste qui-quadrado ou teste de Fischer, e, para as variaveis continuas, o teste de Mann-Whitney foi utilizado. A mediana da idade foi 20,3 anos (15-23) e o evento clinico mais frequente foi o episodio doloroso agudo (73,5%). O tempo medio de permanencia hospitalar foi maior nas internacoes por causas distintas do episodio doloroso agudo (p<0,001), e naquelas com o diagnostico de insuficiencia renal cronica (p=0,006) ou infeccao bacteriana (p=0,002). O numero de obitos foi maior nas internacoes com o diagnostico de infeccao bacteriana (p=0,049) ou insuficiencia renal cronica (p=0,014). Os germes gram-negativos isolados nos pacientes com febre incluiram Pseudomonas sp e Acinetobacter sp. O presente estudo permitiu um maior conhecimento acerca da morbi-mortalidade entre adolescentes e adultos hospitalizados com doenca falciforme. Como poucos estudos sobre internacoes hospitalares estao disponiveis, os achados podem ser uteis no campo da saude publica, em especial na area de planejamento de saude da populacao de pacientes com doenca falciforme.

Factors associated with hospital readmission in sickle cell disease

BackgroundSickle cell disease is the most frequent hereditary disease in Brazil, and people with the disease may be hospitalised several times in the course of their lives. The purpose of this study was to estimate the hazard ratios of factors associated with the time between hospital admissions.MethodsThe study sample comprised all patients admitted, from 2000 to 2004, to a university hospital in Rio de Janeiro State, south-east Brazil, as a result of acute complications from sickle cell disease (SCD). Considering the statistical problem of studying individuals with multiple events over time, the following extensions of Coxs proportional hazard ratio model were compared: the independent increment marginal model (Andersen-Gill) and the random effects model.ResultsThe study considered 71 patients, who were admitted 223 times for acute events related to SCD. The hazard ratios for hospital readmission were statistically significant for the prior occurrence of vaso-occlusive crisis and development of renal failure. However, analysis of residuals of the marginal model revealed evidence of non-proportionality for some covariates.Conclusionthe results from applying the two models were generally similar, indicating that the findings are not highly sensitive to different approaches. The better fit by the frailty model suggests that there are unmeasured individual factors with impact on hospital readmission.

Characterization and analysis of the outcome of adults with acute myeloid leukemia treated in a Brazilian University hospital over three decades

We evaluated the outcome of 227 patients with acute myeloid leukemia during three decades (period 1 - 1980s, N = 89; period 2 - 1990s, N = 73; period 3 - 2000s, N = 65) at a single institution. Major differences between the three groups included a higher median age, rates of multilineage dysplasia and co-morbidities, and a lower rate of clinical manifestations of advanced leukemia in recent years. The proportion of patients who received induction remission chemotherapy was 66, 75, and 85% for periods 1, 2, and 3, respectively (P = 0.04). The median survival was 40, 77, and 112 days, and the 5-year overall survival was 7, 13, and 22%, respectively (P = 0.01). The median disease-free survival was 266, 278, and 386 days (P = 0.049). Survival expectation for patients with acute myeloid leukemia has substantially improved during this 30-year period, due to a combination of lower tumor burden and a more efficient use of chemotherapy and supportive care.

Feasibility and Outcome of the Hyper-CVAD Regimen in Patients With Adult Acute Lymphoblastic Leukemia

BACKGROUND Current chemotherapy regimens for adults with acute lymphoblastic leukemia (ALL) result in high rates of complete remission (CR), but relapses are still frequent. PATIENTS AND METHODS In this retrospective single-center study, we evaluated the results of the Hyper-CVAD (fractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone) regimen in 49 ALL patients treated between 2001 and 2013. No exclusion criteria were applied. The primary outcome measure was the CR rate. RESULTS Forty-six of the 49 patients (93.8%) obtained CR, and 3 (6%) patients died during induction. Philadelphia chromosome was present in 6 patients (12.2%); in all a CR was obtained. Among the 46 patients in CR, 30 (65.2%) received the full planned intensive-phase treatment (8 cycles). Allogeneic hematopoietic cell transplantation was performed in 2 (4%) patients in first CR and in 3 (6%) patients after a second CR. Nonrelapse mortality was observed in 8 patients (16.3%). The median overall survival (OS) and 5-year OS were 24.4 months and 35%, respectively. Initial leukocyte count (> 30 × 10(9)/L) was an important prognostic factor. CONCLUSION Hyper-CVAD as an induction regimen for adults and adolescents with ALL was feasible and yielded a high rate of CR. Relapse rates and OS were comparable to other series but still unsatisfactory.

Daunorubicin 90 mg/m2 in Acute Myeloid Leukemia Induction: Increased Toxicity in Young Patients

Background: The combination of an anthracycline and cytosine arabinoside has been the standard induction therapy for acute myeloid leukemia for more than 3 decades. The clinical benefit of intensification of the daunorubicin dose to 90 mg/m2 has been supported by randomized trials. Based on these promising results, in 2010 we changed our induction protocol of acute myeloid leukemia, increasing the dose of daunorubicin. Patients and Methods: We retrospectively analyzed the treatment outcome of patients treated with high‐dose daunorubicin (90 mg/m2 on days 1‐3) and cytosine arabinoside (200 mg/m2/day continuous infusion on days 1‐7) compared with patients receiving 45 to 60 mg/m2 of daunorubicin. Twenty‐six previously untreated patients younger than 60 years of age were included. Twelve received high‐dose daunorubicin (HD) and 14 the low‐dose (LD). Seventeen patients were in complete remission after 1 induction cycle. Results: There was no overall difference in complete remission rate between HD and LD (66% vs. 64%; P = 1.0). Thirty‐day induction mortality was 15.3% overall, with a nonsignificant difference between groups (25% vs. 7.1%; P = .3). Relapses were observed in 9 (53%) patients: 3 (37.5%) in the HD group and in 6 (66.6%) in the LD group (P = .34). Invasive fungal disease (41.6% vs. 0%; P = .012), creatinine elevation (P = .001), abdominal pain (33% vs. 0%; P = .033), and need for intensive care unit admission (33.3% vs. 0%; P = .033) were more frequent in the HD group. Four patients in the HD group developed neutropenic enterocolitis (P = .033). Conclusion: These data indicate that 90 mg/m2 of daunorubicin increased the toxicity compared with lower doses. &NA; We evaluated the outcome of AML patients treated with daunorubicin 90 mg/m2. Higher rates of invasive fungal disease, antifungal therapy, creatinine elevation, gastrointestinal toxicity and intensive care unit admissions were observed with increased anthracycline dose. The daunorubicin induction dose should be greater than 45 mg/m2 but escalation to 90 mg/m2 seems excessive.