Reticulocyte parameters and hemoglobin F production in sickle cell disease patients undergoing hydroxyurea therapy

Borba, Ronaldo; Lima, C.S.P.; Grotto, Helena Zerlotti Wolf

doi:10.1002/jcla.10070

Cited by 19 publications

(20 citation statements)

References 31 publications

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“…In our study of a Brazilian population we detected a statistical correlation between HbF levels and PCV values, agreeing with the findings of a Nigerian study carried out by Falusi and Kulozik (1990). Borba et al (2003) found no association between the levels of HbF and Hb in Brazilians with SCA who were undergoing treatment with hydroxyurea, although their study did indicate a significant correlation between MCV and HbF levels. Buchanan et al (2004) suggested an association between high HbF levels and a low frequency of leg ulcers, supporting our observation that in our group of Brazilians outpatients with high HbF values had milder SCA phenotypic characteristics and needed fewer sessions of erythrocyte transfusions.…”

Section: Discussionsupporting

confidence: 91%

Clinical and molecular characteristics of sickle cell anemia in the northeast of Brazil

Adôrno

Zanette²,

Lyra³

et al. 2008

Genet. Mol. Biol.

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Beta S-globin gene (β S -globin) haplotypes, markers for severe sickle cell anemia (SCA), and the alpha-thalassemia 2 gene 3.7 kb deletion (-α 2 3.7 kb thal) along with demographic and clinical data were investigated in SCA outpatients (n = 125, 63 female and 62 male) in the Brazilian state of Bahia, which has a high prevalence SCA. PCR-RFLP showed that the Central African Republic/Benin (CAR/BEN, 51.2%) haplotype was most frequent, followed by the Benin/Benin (Ben/Ben, 28.8%). At least one CAR haplotype was present in every outpatient with a history of cerebrovascular accident. The Cameroon (Cam), Senegal (Sen) and Arab-India haplotypes occurred in small numbers, as did atypical haplotypes. Fetal hemoglobin (HbF, %) was unevenly distributed. Compared to those > 18 y, those aged ≤ 18 y had had fewer erythrocyte transfusions and high HbF levels (12.3% ± 7.01 to 7.9% ± 4.36) but a higher frequency of spleen sequestration and pneumonia. Compared with normal α -genes carriers values, the outpatients with -α 2 3.7 kb thal (determined by PCR analysis) had significantly higher mean hemoglobin concentration (Hb) (8.3 ± 1.34 g/dL, p = 0.018) and packed cell volume (PCV = 27.1% ± 4.26, p = 0.019) but low mean corpuscular volume (MCV = 86.1 fL = 10 -15 L ± 9.56, p = 0.0004) and mean corpuscular hemoglobin (MCH = 26.6% ± 4.60, p = 0.039).

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Section: Discussionsupporting

confidence: 91%

Clinical and molecular characteristics of sickle cell anemia in the northeast of Brazil

Adôrno

Zanette²,

Lyra³

et al. 2008

Genet. Mol. Biol.

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“…In our study, patients treated with HU presented increased MCV, MCH, total leukocyte count, and a significant decrease in platelet count compared to untreated patients. Permanence of high MCV, even after the use of HU, was also observed in sickle-cell patients using HU in previous studies (3,17,22) . The HU has also been associated with a decrease in platelet count, total leukocyte reduction and MCH, however, only the first result was observed in our study (5,6,20) .…”

Section: Discussionsupporting

confidence: 64%

Methemoglobin measure in adult patients with sickle-cell anemia: influence of hydroxyurea therapy

Laurentino¹,

Carvalho²,

Santos

et al. 2014

Jornal Brasileiro De Patologia E Medicina Laboratorial

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Introduction: Hemoglobin S (HbS) is unstable hemoglobin that easily oxidizes, causing methemoglobin (MetHb) increased production in patients with sickle-cell anemia (SCA). Objectives: To determine MetHb levels and the influence of hydroxyurea (HU) therapy on this marker in patients with SCA. Materials and methods: Blood samples from 53 patients with SCA at the steady-state, with and without HU therapy, and 30 healthy individuals were collected to evaluate MetHb levels. The MetHb measurement was performed by spectrophotometry. Complete blood count, HU measurements, and fetal hemoglobin (HbF) and HbS concentrations were taken from medical records. Results: MetHb levels were statically higher in patients with SCA when compared to control group (p < 0.001). There was no statistical difference in MetHb level between SCA patients, either using or not HU. We obtained a positive correlation between MetHb measurements and HbS concentration (r = 0.2557; p = 0.0323). Conclusion: HbS presence favored hemoglobin breaking down, and consequently increased MetHb production. Treatment with HU, however, did not influence the levels of this marker.

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“…(17) The MCV of patients in this study after starting treatment with HU increased significantly compared to before treatment. Thus, as there was a significant correlation between MCV and Hb F, it can be stated that in this study the increased Hb F is directly related to the increase of cell volume.…”

Section: Discussionmentioning

confidence: 68%

Patients with sickle cell disease taking hydroxyurea in the Hemocentro Regional de Montes Claros

Santos

Maia

2010

Rev. Bras. Hematol. Hemoter.

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BackgroundThe development of therapies for sickle cell disease has received special attention, particularly those that reduce the polymerization of hemoglobin S. Hydroxyurea is a commonly used medication because it has the ability to raise levels of fetal hemoglobin, decrease the frequency of vaso-occlusive episodes and thus improve the clinical course of sickle cell disease patients.ObjectiveTo study hematological data and the clinical profile of sickle cell disease patients taking hydroxyurea in a regional blood center.MethodsFrom the charts of 20 patients with sickle cell anemia, the clinical outcomes and a number of hematological variables were analyzed before and during treatment with hydroxyurea.ResultsThe patients' ages ranged from 6 to 41 years old, most were dark skinned and there was a predominance of women. The main symptom that defined whether patients were prescribed hydroxyurea was painful crises followed by hospitalizations. During treatment with hydroxyurea there were significant increases in hemoglobin, fetal hemoglobin, mean corpuscular volume and mean corpuscular hemoglobin. The reticulocyte and white blood cell counts dropped significantly with treatment. A positive correlation was found between fetal hemoglobin and mean corpuscular volume before and during treatment. Additionally, a correlation was found between the white blood cell and reticulocyte counts before treatment with hydroxyurea.ConclusionMost patients showed improvements with treatment as demonstrated by increases in hemoglobin, fetal hemoglobin and mean corpuscular volume, as well as by reductions in the reticulocyte and white blood cell counts. Clinically, more than 50% of patients had a significant reduction of events.

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Reticulocyte parameters and hemoglobin F production in sickle cell disease patients undergoing hydroxyurea therapy

Cited by 19 publications

References 31 publications

Clinical and molecular characteristics of sickle cell anemia in the northeast of Brazil

Clinical and molecular characteristics of sickle cell anemia in the northeast of Brazil

Methemoglobin measure in adult patients with sickle-cell anemia: influence of hydroxyurea therapy

Patients with sickle cell disease taking hydroxyurea in the Hemocentro Regional de Montes Claros

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