Christina Matzenbacher Bittar

High prevalence of anemia in children and adult women in an urban population in southern Brazil.

This population-based study was designed to detect the prevalence of anemia in a healthy population of children (18 months to 7 years) and women (14 to 30 years) tested in 2006–2007 in the state of Rio Grande do Sul, Brazil as part of an effort to tackle this massive problem that still affects so many people in the XXI century. Anemia was defined according to the WHO. Capillary blood was measured and socioeconomic status was determined according to the Brazilian Association of Market Research Agencies. The median prevalence of anemia in 2198 children was 45.4% and in 1999 women 36.4%. Anemia decreased with age during childhood; although significantly more prevalent in lower classes individuals, it was also high in the upper classes. There are indirect evidences that the lack of iron supplementation and/or iron fortified food may play a role in it. Professionals and society wise measures of education have to be implemented in order to address possible biologic factors involved in childhood psychosocial development in southern Brazil.

The expression of CD56 antigen is associated with poor prognosis in patients with acute myeloid leukemia

Background The expression of CD56 is considered a bad prognostic factor for overall survival, lower rates or short complete remission and extramedullary invasion but the results are controversial. The importance of validating new prognostic parameters in acute leukemias was the reason to investigate the CD56 expression in blast cells of patients with acute myeloid leukemia. Methods A cohort of 48 patients treated at Hospital de Clinicas de Porto Alegre and diagnosed with acute myeloid leukemia as classified by the French-American-British group (FAB) criteria using cell morphology, cytochemistry and flow cytometry were evaluated. Results Eight cases (16.7%) were CD56 positive without correlation to age or gender. The highest incidence of CD56 positivity was in FAB subtypes M4 and M5. The death rate during induction was not significantly different between patients with and without CD56 expression (62.5% vs. 27.5%; p-value = 0.097). However, patients that expressed CD56 had significantly lower overall survival than those who did not (mean 4.0 months vs. 14.5 months; p-value = 0.03). Conclusions The data suggest that expression of CD56 in acute myeloid leukemia may be indicative of poor prognosis because it is associated with a shorter overall survival. The death rate during induction was not significantly different despite an apparent difference in proportions between groups.

Length of treatment and dose as determinants of mutagenicity in sickle cell disease patients treated with hydroxyurea.

Hydroxyurea (HU) is an antineoplastic drug widely used in the clinical management of patients with sickle cell disease (SCD), and many questions related with its use remain unresolved. Given the severity of SCD, HU benefits, although not thoroughly confirmed, seem to outweigh its potential carcinogenicity. This study aimed to assess the genotoxicity associated with HU dose and treatment length by evaluating mutagenicity in patients with SCD treated with HU (SCHU) using the cytokinesis-block micronucleus assay (CBMN) in white cells. The study was conducted with 35 individuals in the SCHU group and 34 controls matched according to age, sex and smoking habit. CBMN results showed an increase (p=0.032) in the number of micronuclei (MN), but not of nucleoplasmic bridges (NPB) or nuclear buds (NBUD) in the SCHU group. The increased frequency of MN in the SCHU group was significantly correlated with treatment length and final HU dose, which confirms that patients with SCD treated with HU should be carefully monitored to reduce the risk of carcinogenicity.

Prevalência de talassemias e hemoglobinas variantes em pacientes com anemia não ferropênica

To establish the frequency of hemoglobinopathies and thalassemias in patients with non-ferropenic anemia, 58 patients with confirmed non-ferropenic anemia and 235 non-anemic individuals (control group) were studied. All samples were obtained from the Hospital de Clinicas de Porto Alegre (HCPA), Rio Grande do Sul, Brazil. The techniques used were Alkaline pH cellulose acetate electrophoresis and cytological screening of Hb, Hl, HPLC, hemogram and ferritin. The data analysis showed that 63% of the patients with non-ferropenic anemia carried some type of inherited anemia: 25.9% of heterozygous a-thalassemia, 32.8% of heterozygous b-thalassemia, 3.4% of heterozygosity for hemoglobin S (Hb AS) and 1.7% of homozygosity for hemoglobin C (Hb CC). Inherited anemias were detected in 14.1% of the control group: 11.5% of a-thalassemia, 0.9% of b-thalassemia, 1.3% of heterozygosity for hemoglobin S (Hb AS) and 0.4% of heterozygosity for hemoglobin C (Hb AC). The results obtained showed that the prevalence of variant thalassemias and hemoglobins in the control group is coincident with that described in the literature. However, physicians and health services should be informed about the overwhelming prevalence of these inherited homeopathies in individuals with non-ferropenic anemia, due to its importance in the definitive diagnosis of anemia and for the correct therapeutic proceedings.

Leucemia mielomonocítica juvenil: relato de caso

A leucemia mielomonocitica juvenil (LMMJ) e uma doenca rara, que representa de 2%a 3% de todas as leucemias pediatricas. E uma doenca clonal de celulas da linhagem mieloide, que apresenta caracteristicas de mieloproliferacao e de displasia. Os sinais e os sintomas sao resultantes da infiltracao de celulas monociticas malignas em orgaos nao hematopoeticos. Os sintomas mais comuns sao febre, tosse, infeccao, fraqueza, palidez, linfadenopatia, hepatoesplenomegalia, lesoes cutâneas e manifestacoes hemorragicas. Como a LMMJ exibe um curso clinico muito agressivo e responde pobremente a quimioterapia, o transplante de celulas-tronco hematopoeticas e a unica modalidade terapeutica curativa. Neste estudo, relatamos o caso de um paciente do sexo masculino, com um ano e dez meses de idade, que compareceu na emergencia do Hospital de Clinicas de Porto Alegre por apresentar febre, com diagnostico previo de mononucleose feito em outra Instituicao. A apresentacao clinica, em conjunto com os achados laboratoriais, permitiu o diagnostico correto. O paciente foi tratado com quimioterapia e submetido a transplante de celulas-tronco hematopoeticas.

Long-term follow-up of kidney allografts in patients with sickle cell hemoglobinopathy

Embora a anemia falciforme e as sindromes falciformes frequentemente causem varias alteracoes funcionais renais, nao e comum a insuficiencia renal terminal. Nestes casos, o transplante renal e uma alternativa que se acompanha de resultados comparaveis aos obtidos em receptores sem hemoglobinopatias. Esta estrategia terapeutica tem sido, no entanto, pouco relatada para portadores de hemoglobinopatia SC. Este relato descreve a evolucao de dois pacientes portadores de hemoglobinopatia SC que foram submetidos ao transplante renal. No momento do transplante ambos apresentavam severa anemia e crises dolorosas frequentes. Os pacientes evoluiram com boa funcao do enxerto, parâmetros hematologicos quase normais e praticamente assintomaticos do ponto de vista da hemoglobinopatia, treze e oito anos apos o transplante. Estes casos ilustram que a insuficiencia renal terminal causada pela hemoglobinopatia SC pode ser tratada com sucesso pelo transplante renal, nao so do ponto de vista renal, propriamente dito, mas tambem em termos de sua doenca hematologica.