Rodrigo Portugal

Risk Factors for Invasive Fusariosis in Patients With Acute Myeloid Leukemia and in Hematopoietic Cell Transplant Recipients

BACKGROUND Risk factors for invasive fusariosis (IF) have not been characterized. We attempted to identify risk factors for IF in a prospective cohort of hematologic patients treated in 8 centers in Brazil. METHODS Patients with (cases) and without (controls) proven or probable IF diagnosed in a cohort of patients with acute myeloid leukemia (AML) or myelodysplasia (MDS), and in allogeneic hematopoietic cell transplant (HCT) recipients (early, until day 40; late, after day 40 posttransplant) were compared by univariate Cox regression analysis. RESULTS Among 237 induction remission courses of AML/MDS and 663 HCTs (345 allogeneic and 318 autologous), 25 cases of IF were diagnosed. In the AML/MDS cohort, active smoking (hazard ratio [HR], 9.11 [95% confidence interval {CI}, 2.04-40.71]) was associated with IF. Variables associated with IF in the early phase of allogeneic HCT were receipt of antithymocyte globulin (HR, 22.77 [95% CI, 4.85-101.34]), hyperglycemia (HR, 5.17 [95% CI, 1.40-19.11]), center 7 (HR, 5.15 [95% CI, 1.66-15.97]), and AML (HR, 4.38 [95% CI, 1.39-13.81]), and in the late phase were nonmyeloablative conditioning regimen (HR, 35.08 [95% CI, 3.90-315.27]), grade III/IV graft-vs-host disease (HR, 16.50 [95% CI, 2.67-102.28]), and previous invasive mold disease (HR, 10.65 [95% CI, 1.19-95.39]). CONCLUSIONS Attempts to reduce the risk of IF may include smoking cessation, aggressive control of hyperglycemia, and the use of a mold-active agent as prophylaxis in patients receiving nonmyeloablative HCT or ATG in the conditioning regimen. Future research should further explore smoking and other prehospital variables as risks for IF.

Clinical Factors Predictive of Bone Marrow Involvement in Hodgkin's Disease

The role of bone marrow biopsy in the staging of Hodgkins disease is undergoing reevaluation. We have studied the relationship of clinical factors to the presence of bone marrow involvement in 130 previously untreated patients with Hodgkins disease. The presence of fever, spleen enlargement, anemia, leukopenia, poor performance status and poor histologic subgroups were positively correlated with the presence of bone marrow involvement in the univariate analysis. In the multivariate analysis, only fever, spleen involvement, leukopenia and poor histologic subgroups were significant. The predictive value of the absence of fever in regard to the absence of bone marrow involvement was 98%. The likelihood of bone marrow involvement in the absence of all four significant factors was only 0.05%. Patients without these clinical factors should probably not be submitted to a bone marrow biopsy as part of the staging procedures performed in Hodgkins disease.

A simple score to predict early death in adult cancer patients with bloodstream infections.

OBJECTIVE To develop a simple score to predict early death in adult cancer patients with bloodstream infection. METHODS A prediction score was developed by analyzing data collected from 361 episodes of BSI at an oncology cancer care center in Brazil. Early death was defined as all deaths occurring within 7 days of the first positive blood culture. RESULTS Multivariate regression analysis identified poor Karnofsky performance status, uncontrolled cancer, hypotension, pulmonary infiltrates, associated infectious sites, and neutropenia as independently associated with death. Predictive scores were developed assigning points to each significant independent variable. The overall mortality was 20.5% and, the total weighted score ranged from 0 to 11 points, with a very good calibration (Hosmer-Lemeshow statistic, P=0.92) and discrimination (area under receiver operating characteristic curve=0.893). The cutoff value for the predictive score was 3 points, with a negative predictive value of 99% and sensitivity of 98.6%. CONCLUSION The score model was able to identify adult cancer patients with bloodstream infection at lower risk for early death with an elevated degree of certainty as depicted by a very high negative predictive value. It is essential to prospectively validate the rule in a different group of patients.

Thiabendazole for the Treatment of Strongyloidiasis in Patients with Hematologic Malignancies

A total of 21 patients with hematologic malignancies were given thiabendazole for treatment of strongyloidiasis. Fifteen patients were cured. Since there were no relapses, it is unlikely that maintenance therapy has a role in the management of strongyloidiasis in this population of patients.

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.

Cutaneous granulocytic sarcoma in myelodysplastic syndrome

Granulocytic sarcoma is an extramedullary tumor of immature cells of granulocytic series, generally associated to acute myelogenous leukemia. The skin is one of the most commonly affected sites. Granulocytic sarcoma can complicate myelodysplastic syndromes and is considered a sign of poor prognosis. They are often misdiagnosed with non‐Hodgkin lymphoma of the lymphoblastic type, Burkitt lymphoma and large cell lymphoma. In children, the differential diagnoses also include small, round cell tumors. It is important to diagnose these lesions early because they can precede peripheral blood and bone marrow transformation to acute myelogenous leukemia. We report a case of an elderly patient with myelodysplastic syndrome who developed multiple cutaneous granulocytic sarcoma lesions and discuss prognostic and treatment implications.

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.

Hematologic neoplasias and acromegaly

We report a 59-year-old acromegalic woman, who presented with generalized bone pain, weakness, fatigue and foamy urine, who was found to have multiple myeloma (MM); and a 60-year-old acromegalic woman with dizziness, vomiting and abdominal pain, high blood pressure and splenomegaly that was posteriorly diagnosed as having Waldenstrom’s macroglobulinemia (WM). Acromegaly is an uncommon disease and epidemiological studies have provided increasingly debated evidence that elevated IGF-I levels might enhance the neoplastic risk, and that cancers constitute the third leading cause of mortality in acromegaly. It is known that GH and IGF-I can activate B cell lymphocytes, and that IGF-I receptor is universally expressed in MM cells. Although the complication of acromegaly with WM or MM in patients has rarely been reported until now, we described two case reports of acromegalic patients with those hematological neoplasias, which allow a discussion about this controversial issue.

Invasive fungal diseases in patients with acute lymphoid leukemia

Abstract Invasive fungal disease (IFD) represents an important complication in patients with acute lymphoid leukemia (ALL). The objectives of this study were to determine the prevalence of IFD in ALL patients with neutropenia, identify factors associated with IFD, and estimate the impact of IFD on the outcome. All patients with ALL who developed febrile neutropenia from 1987 to 2013 were evaluated. Cases of IFD were classified as proven or probable. Factors associated with IFD were evaluated by comparing episodes with and without a diagnosis of IFD. Among 350 episodes of febrile neutropenia, 31 IFDs were diagnosed (8.8%). Prolonged neutropenia was the only factor associated with IFD caused by yeasts. Factors associated with IFD caused by molds by multivariate analysis were the period after 2008, receipt of allogeneic transplant, relapsed ALL and prolonged neutropenia. Patients in relapse should receive induction chemotherapy in rooms with HEPA filter and receive antifungal prophylaxis.