Hemoglobin Willamette (ß51Pro → Arg): Case Report and Literature Review.

We report a case of hemoglobin (Hb) Willamette (ß51 Pro → Arg) in the Hematology Department of a tertiary hospital in Fortaleza, Northeast of Brazil. A literature review of the cases described in health sciences databases using as a descriptor Hb Willamette was performed, revealing 12 reported cases, of which only one presented with anemia. Herein, we describe a case of a female 29 years old, with hemoglobinopathy Willamette presenting clinically with anemia, having the lowest hemoglobin rate of the published cases. The relatives of the patient were evaluated andthe patient's mother corresponded to the first description of the association between Hb Willamette and HbC. Among the hemoglobinopathies, hemoglobin Willamette is an extremely rare disease; therefore it is important to analyze its clinical and laboratory manifestations for accurate diagnosis and assessment of potential interactions with other genetic variants.

Adherence to treatment with imatinib in chronic myeloid leukemia: a study of the first decade of responses obtained at a Brazilian hospital.

OBJECTIVE: The aim of this study was to identify the reasons for failure in adherence to imatinib mesylate treatment in chronic myeloid leukemia. METHODS: A retrospective review was performed of 100 non-electronic records of patients with Ph(+) chronic myeloid leukemia treated with imatinib mesylate. The study period was from January 2001 to January2011. Data were analyzed by Chi-Square and Correspondence analysis using the Statistical Analysis System software package. RESULTS: At the beginning of treatment 41% of patients were in advanced stages of the disease. The unavailability of the drug (44.8%) and myelotoxicity (25.7%) were the most frequent reasons for interruption. The adherence rate was < 90% in 47% of the cases. The low adherence influenced the cytogenetic response (p-value = 0.020) and molecular response (p-value = 0.001). Very high adherence (> 95%) induced complete cytogenetic response, major cytogenetic response and major molecular response. CONCLUSION: The population of this study obtained lower-than-expected therapeutic responses compared to other studies.

Study of correlation of nitrite levels with malonaldehyde and the prognosis of patients with sickle cell disease on hydroxyurea, Ceará-Brazil.

Hydroxyurea (HU) is the only drug approved for the induction of fetal hemoglobin. Besides this benefit, there are others such as the reduction of leukocyte and generation of nitric oxide (NO). Sickle cell anemia (SCA) is characterized by chronic hemolytic anemia and vaso-occlusive phenomena. The aim of this study was to evaluate the correlation of parameters MDA and NO2 with the prognosis of patients with SCA as outpatients at Hospital Universitário Walter Cantídeo. In all, 65 patients with SCA--51 without the use of HU (group I) and 14 chronically treated with HU (group II)--were recruited. Nitrite and malonaldehyde were determined by biochemical methods. We found that in group II there was a significant difference of serum MDA with clinical variables: two or more transfusions during the year (P<0.0469), the presence of malleolar ulcers (P<0.0400), and the occurrence of vaso-occlusive episodes (P<0.0031), and Group I with the occurrence of three or more vaso-occlusive episodes (P<0.0051). Correlating the malonaldehyde with clinical variables in groups I and II, we observed a statistically significant relationship with two or more transfusions during the year and the presence of malleolar ulcer. Our results demonstrate that MDA levels can be used as parameter for prognosis in SCA.