Thalassemia and the Hematology of Homozygous Sickle Cell Disease in Childhood

نویسنده

  • H. Teal
چکیده

a Thalassemia modifies the hematologic expression of homozygous sickle cell (55) disease, resulting in increased total hemoglobin and HbA2 and decreased HbF. mean cell volume, reticulocytes. irreversibly sickled cells. and bilirubin levels. The age at which these changes develop in children with 55 disease is unknown. Ascertainment of globin gene status in a large representative sample of children with 55 disease has afforded an opportunity to study the hematologic indices in nine children homozygous for a thalassemia 2 (two-gene group). 90 children heterozygous for a thalassemia 2 (three-gene group). and 167 children with a normal a globin gene complement (fourgene group). The two-gene group had significantly lower a Thalassemia 2 is common in black populations, heterozygotes and homozygotes occurring in -30% and 4%, respectively, ofiamaican infants’ and black Americans.2 Inheritance of a thalassemia in homozygous sickle cell (55) disease ameliorates the hematologic features, 7 although observations of clinical benefit are less consistent.4’6 Among older children and adults, homozygous a thalassemia 2 resulted in higher levels of total hemoglobin, hematocrit, red cell count, and HbA2, and lower levels of HbF, mean cell volume, reticulocytes, and irreversibly sickled cells. These findings were compatible with reduced hemolysis, which was supported by a prolonged red cell survival.8 Whether these changes occur in young children is unknown, as is the age at which the changes develop. In the Jamaican sickle cell cohort study, the effects of a thalassemia on the development of hematologic changes in 55 disease from birth have been observed.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Correlation between Nitric oxide (NO) & Asymmetric dimethylargininie (ADMA) Hemoglobin

73/ Spring summer 2010, Vol.4, No. 1 Medical Laboratory Journal Correlation between Nitric oxide (NO) & Asymmetric dimethylargininie (ADMA) Hemoglobin Concentration in sickle cell patients Abstract Background and objectives: The importance of Nitric oxide (NO) and Asymmetric dimethylargininie (ADMA) in pathophysiology of Sickle cell disease (SCD) is being increasingly clarified. Since very few ...

متن کامل

Pulmonary Spirometry Parameters in Patients with Sickle Thalassemia and Sickle Cell Disease at Shafa Hospital in Khuzestan Province-Iran

Abstract Background Prevalence of hereditary blood diseases such as sickle cell anemia, sickle thalassemia and thalassemia major are high in Khuzestan province. Sickle cell anemia and beta-thalassemia are predominantly common in Iranian Arabs. Pulmonary complications account for a large proportion of morbidity and mortality in patients with and sickle cell disease. Periodic lung function asse...

متن کامل

Genotyping of Individuals with Hemoglobinopathies in Beja Tribes and Other Minor Groups in Port Sudan, Eastern Sudan

ABSTRACT             Background and objectives: This study aimed to characterize the spectrum of β-thalassemia mutations and haplotypes of sickle cell anemia in Beja tribes and other minor groups living in Port Sudan, Sudan.             Methods: This descriptive cross-sectional study was carried out from March 2011 to July 2013. Overall, 209 anemic patients were screened for hemoglobinopathy ...

متن کامل

Level of Hemoglobin F and Gg Gene Expression in Sickle Cell Disease and Their Association with Haplotype and XmnI Polymorphic Site in South of Iran

Background: Molecular genetic factors regulating hemoglobin F (Hb F) expression are important modifiers of the severity of sickle cell anemia (SS). Methods: The prevalence of XmnI polymorphic site, the Gg:Ag ratio and the Hb F level were determined using PCR-RFLP procedure, HPLC and alkaline denaturation method, respectively, in various haplotypes of 52 patients with SS, 18 patients with sickle...

متن کامل

Can Trimodal Distribution of HbS Levels in Sickle Cell Traits Be Used To Predict the Associated Alpha-Thalassemia For Screening Cases in Central India?

Background: Until now, trimodal distribution of HbS has been seen by six different studies in the world when associated with alpha-thalassemia with confirmation by corresponding alpha-genotyping studies. The RBC indices reduce as alpha-globin genes reduce in sickle cell trait (SCT) patients, which decreases the extent of intra-vascular sickling and thus betters the clinical cou...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره   شماره 

صفحات  -

تاریخ انتشار 2005