Leukemoid reaction: A 21st-century cohort study.

INTRODUCTION: Leukemoid reaction (leukocyte count >50 cells ×109  L) is a rare but extremely relevant finding. Since little has been published on this condition's clinical relevance and prognosis, we investigated leukemoid reaction in patients with a white blood cell count of >50 × 109  L, including etiology and outcomes. METHODS: This retrospective cohort study included all patients at a Brazilian tertiary hospital between January 2016 and July 2018 > 18 years with a total leukocyte count >50 cells×109  L. Demographics, complete blood count, clinical features, and the exams used to diagnose and determine leukemoid reaction etiology were analyzed. A Kaplan-Meyer survival analysis was performed, and a binary logistic regression model identified variables associated with death. RESULTS: Of the 267 cases with white blood cell count of >50 × 109 , 162/267 (60%) were secondary to hematopoietic neoplasm and 105/267 (40%) presenting as a true leukemoid reaction. The primary causes of the true leukemoid reaction cases were infection (59), nonhematopoietic neoplasm (17), or other causes (29). Patient deaths (66) differed significantly between groups (P < .001, log-rank [Mantel-Cox] Test). Lower hemoglobin, older age, and increased segmented neutrophil count were associated with increased risk of death. CONCLUSIONS: This was a modern cohort analysis of leukemoid reactions, inclusive of all etiologies. The most common cause was infection, which involved several microorganisms. Paraneoplastic leukemoid reaction was also common. Both conditions have a poor prognosis with high mortality, being a major medical challenge.

Hepcidin is a useful biomarker to evaluate hyperferritinemia associated with metabolic syndrome.

Investigation of hyperferritinemia in metabolic syndrome patients represents a diagnostic challenge, but it is essential for the identification of individuals with iron overload. Hepcidin negatively regulates iron absorption and release. An increase in hepcidin occurs when iron levels are sufficient or in inflammatory states, conditions often associated with hyperferritinemia. Hemochromatosis causes hyperferritinemia due to iron overload, but frequently has low hepcidin levels. Our aim was to evaluate biochemical and molecular parameters related to iron metabolism in patients with metabolic syndrome. We evaluated 94 patients with metabolic syndrome according to the International Diabetes Federation criteria in a cross-sectional study. Anthropometric data and diagnostic criteria for metabolic syndrome, iron dosage, ferritin, transferrin saturation, hepcidin, and the C282Y and H63D mutations in the HFE hemochromatosis gene were evaluated. Prevalence of hyperferritinemia in the study population was 27.7% and was higher in males (46.2%) than in females (14.5%). Increase in transferrin saturation correlated with mutations in the hemochromatosis gene. Hyperferritinemia was associated to transferrin saturation and hepcidin after logistic regression analysis. In conclusion, hyperferritinemia is a frequent finding in metabolic syndrome patients, most frequently in men; and hepcidin assessment can be useful for the investigation of ferritin increase in those subjects.

Prevalence of low bone mineral density in adolescents and adults with cystic fibrosis.

OBJECTIVE: The aim of this cross-sectional study was to evaluate the prevalence of low bone mass density in cystic fibrosis patients as well as to evaluate the factors associated with bone mass in such patients. METHODS: Bone mass density was measured by dual-photon X-ray absorptiometry of lumbar spine (L1-L4), in patients ≤19 years old, or lumbar spine and femur (total and neck) in patients ≥20 years old. Evaluations of nutritional status, biochemical parameters, and lung function were performed. Medication data were obtained from medical records. RESULTS: Fifty-eight patients were included in the study (25 males/ 33 females), mean age 23.9 years (16-53 years). The prevalence of bone mass below the expected range for age at any site was 20.7%. None of the subjects had history of fracture. Lumbar spine Z-score in cystic fibrosis patients correlated positively with body mass index (r= 0.3, p=0.001), and forced expiratory volume in the first second (% predicted) (r=0.415, p=0.022). Mean lumbar spine Z-score was higher in women (p=0.001), in patients with no pancreatic insufficiency (p=0.032), and in patients with no hospitalization in the last 3 months (p=0.02). After multivariate analysis, body mass index (p= 0.001) and sex (p=0.001) were independently associated with Z-score in lumbar spine. CONCLUSION: Low bone mass is a frequent problem in patients with CF, being independently associated with body mass index, and male sex.

Prevalence of low bone mineral density in adolescents and adults with cystic fibrosis / Prevalência de densidade mineral óssea baixa em adolescentes e adultos com fibrose cística

Objective The aim of this cross-sectional study was to evaluate the prevalence of low bone mass density in cystic fibrosis patients as well as to evaluate the factors associated with bone mass in such patients. Methods Bone mass density was measured by dual-photon X-ray absorptiometry of lumbar spine (L1-L4), in patients ≤19 years old, or lumbar spine and femur (total and neck) in patients ≥20 years old. Evaluations of nutritional status, biochemical parameters, and lung function were performed. Medication data were obtained from medical records. Results Fifty-eight patients were included in the study (25 males/ 33 females), mean age 23.9 years (16-53years). The prevalence of bone mass below the expected range for age at any site was 20.7%. None of the subjects had history of fracture. Lumbar spine Z-score in cystic fibrosis patients correlated positively with body mass index (r= 0.3, p=0.001), and forced expiratory volume in the first second (% predicted) (r=0.415, p=0.022). Mean lumbar spine Z-score was higher in women (p=0.001), in patients with no pancreatic insufficiency (p=0.032), and in patients with no hospitalization in the last 3 months (p=0.02). After multivariate analysis, body mass index (p= 0.001) and sex (p=0.001) were independently associated with Z-score in lumbar spine. Conclusion Low bone mass is a frequent problem in patients with CF, being independently associated with body mass index, and male sex. .

Objetivo Determinar a prevalência de massa óssea baixa em pacientes adolescentes e adultos com fibrose cística e estudar os fatores potencialmente associados. Métodos Densidade mineral óssea foi determinada por absorciometria por dupla emissão de raios X na coluna lombar em pacientes ≤ 19 anos e na coluna e no fêmur em pacientes ≥ 20 anos. Avaliações nutricionais, bioquímicas e pulmonares foram realizadas. Dados referentes ao tratamento farmacológico foram coletados. Resultados 58 pacientes foram incluídos no estudo (25 homens/33 mulheres), média de idade de 23,9 anos (16-53). Massa óssea abaixo da esperada foi verificada em 20,7% dos pacientes. Não houve histórico de fratura. Z-score da coluna lombar associou-se positivamente com índice de massa corporal (r=0,3; p=0,022), volume expiratório forçado (% previsto) (r=0,415; p=0,001). A média do Z-score da coluna foi mais alta nas mulheres que nos homens (p=0,001), em pacientes que não possuíam insuficiência pancreática (p=0,02) e em pacientes que não haviam sido hospitalizados nos últimos três meses (p=0,032). Os fatores encontrados como preditores independentes de Z-score da coluna lombar foram sexo masculino (p=0,001) e índice de massa corporal (p=0,001). Conclusão Massa óssea baixa é frequente em pacientes com FC, estando associada independentemente com índice de massa corporal e sexo masculino. .

Análise do perfil de solicitações para transfusão de hemocomponentes no Hospital de Clínicas de Porto Alegre no ano de 2005 / Blood-based products request profile analysis in Hospital de Clínicas de Porto Alegre in 2005

O uso de hemocomponentes em hospitais de alta complexidade é elevado, sendo necessário controle e racionalização de sua utilização, dada sua escassez e alto custo. Para tal, muitos hospitais desenvolveram protocolos para uso de hemocomponentes. O objetivo deste trabalho foi descrever as características epidemiológicas das solicitações de hemoderivados e sua adequação em conformidade ao protocolo vigente no Hospital de Clínicas de Porto Alegre. Por meio de estudo retrospectivo transversal, foram avaliadas a freqüência de transfusões e suas indicações no ano de 2005 em três áreas (Internação Clínica, Cirúrgica e Centro de Terapia Intensiva). Analisaram-se as justificativas de cada solicitação em conformidade com o protocolo vigente. Foram identificadas 12.175 solicitações de hemoderivados no ano de 2005. O hemocomponente mais solicitado foi o concentrado de hemácias (6.578 solicitações), seguido do concentrado de plaquetas (4.133), plasma fresco (1.296) e do crioprecipitado (168). As perdas representaram 2,96 por cento dos eventos. As três áreas foram responsáveis por 59,77 por cento das solicitações de hemocomponentes. A internação clínica apresentou 85,57 por cento de solicitações em conformidade com o protocolo, seguida da CTI com 81,4 por cento e da cirurgia com 71,42 por cento. A maioria das solicitações de hemocomponentes esteve de acordo com o protocolo da instituição no ano de 2005. A internação clínica teve o perfil mais adequado de solicitações no ano de 2005, seguida do CTI e, por fim, da internação cirúrgica.

The use of blood-based products in a tertiary hospital is elevated so control and rational use is necessary. Many hospitals have protocols for the use of blood components. The aim of this work was to describe the epidemiological characteristics of blood-based product requests and their adjustment to the protocol of Hospital de Clínicas in Porto Alegre. Using a cross-sectional retrospective study, we analyzed the frequencies and indications of transfusion requests in 2005 in three different sectors (Clinical, Surgical and Intensive Care Unit). Each transfusion request was analyzed according to the current protocol. A total of 12,175 transfusion requests were analyzed. Requests for blood (6,578) were the commonest, followed by platelets (4,133), fresh frozen plasma (1296) and cryoprecipitate (168). Lost data represented 2.96 percent of the total events. The three sectors were responsible for 59.77 percent of blood-based product requests. The Clinical Sector requested 85.57 percent of its transfusions satisfactorily, followed by the ICU (81.4 percent) and finally the Surgical Sector (71.42 percent). The majority of transfusion requests in HCPA in 2005 were adequate according to the protocol. The Clinical Sector had the best request profile, followed by the ICU and the Surgical Sectort.