Lemonne N (2015) Hematologica
Link to abstract: http://www.haematologica.org/content/100/10/e383
Study on 24 adult patients in Guadeloupe with Sickle Cell Anemia: 50% had 1 a-gene deletion, who were given hydroxyurea treatment including escalating doses for history of frequent VOC and or ACS or frequent subclinical VOC and anemia in the preceding year. Outcomes measures: hematocrit (Hct), blood viscosity, red cell deformability and RBC disaggregation threshold as well as clinical VOC and ACS. Blood samples were taken at 1,3,6 and 12 months of HU. Hemoglobin and Hct increased from 3rd month of therapy; RBC deformability increased from 1 month onwards - effects more pronounced in non a-gene deletion. The blood viscosity did not change in SCA patients receiving HU therapy, which is important as increased viscosity increases the frequency of VOC in SCA. Also implications for possible use of HU in other sickle syndromes e.g. SC disease, which have increased blood viscosity compared to SCA.