The haematology of homozygous sickle cell disease after the age of 40 years

Morris, J.N.; Dunn, David; Beckford, M.; Grandison, Yvonne; Mason, Karlene; Higgs, Douglas R.; Ceulaer, Karel De; Serjeant, B. E.; Serjeant, G. R.

doi:10.1111/j.1365-2141.1991.tb08588.x

Cited by 36 publications

(21 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Consistent with the work of Adekile and Haider [24], HbF levels did not differ significantly between male and female SS patients. This may be due to the age of the sickle cell anemia population studied, as differences between the sexes have been described in adults [25]. The most interesting finding of our study is the relationship between HbF levels and age (table 2).…”

Section: Discussionsupporting

confidence: 63%

Sickle-Cell Disease in Brazzaville, Congo: Genetical, Hematological, Biochemical and Clinical Aspects

et al. 1999

View full text Add to dashboard Cite

Clinical, hematological and molecular features of 116 unrelated sickle-cell anemia patients from Brazzaville were investigated. The mean age of the patients was 9.4 ± 5.3 years. 232 β^s-chromosomes were haplotyped and almost all sickle cell anemia patients (91%) were homozygous for the Bantu haplotype. All hematological indices were similar for males and females. Mean hemoglobin (Hb) concentration was 6.6 ± 1.4 g/dl. Fetal Hb (HbF) levels were from 1 to 28%, with a mean of 8.8 ± 5.8%. There was a curvilinear relationship between %HbF and age suggesting that HbF level had a selective effect on the survival of patients. This effect was sex dependent. The (–α^3.7) gene frequency was 0.45 and was not affected by stratification for age. Hematological characteristics of patients with (–α/αα, –α/–α) and without (αα/αα) the –α^3.7 α-thal-2 deletion showed trends similar to those reported in Jamaican and US sickle cell anemia patients. Hyperbilirubinemia (>38 μmol/l) was common and high lactic dehydrogenase (LDH) concentrations were recorded. Bilirubin concentrations for males and females were similar whereas those for LDH concentration were not. Hepatomegaly and splenomegaly were common. Splenomegaly was strongly associated with the –α^3.7 α-thal-2 deletion. These clinical and hematological observations indicate a more severe form of hemolytic disease in Congolese SS patients.

show abstract

Section: Discussionsupporting

confidence: 63%

Sickle-Cell Disease in Brazzaville, Congo: Genetical, Hematological, Biochemical and Clinical Aspects

et al. 1999

View full text Add to dashboard Cite

show abstract

“…It is not possible, however, to exclude that lower platelet counts could be associated with the presence of hepatitis C or older age, as already described in SCD [29,30]. Although our study evidenced older age in this subgroup of patients, no association with hepatitis C could be noted.…”

Section: Discussioncontrasting

confidence: 43%

Pulmonary hypertension diagnosed by right heart catheterisation in sickle cell disease

Fonseca

Souza²,

Salemi³

et al. 2011

European Respiratory Journal

233

224

View full text Add to dashboard Cite

Recent studies have recognised the importance of pulmonary hypertension (PH) in sickle cell disease (SCD). The aim of this study was to determine the prevalence and prognostic impact of PH and its features in patients with SCD.80 patients with SCD underwent baseline clinical evaluation, laboratory testing, 6-min walk tests (6MWTs) and echocardiography. Patients with a peak tricuspid regurgitant jet velocity (TRV) of o2.5 m?s -1 were further evaluated through right heart catheterisation (RHC) to assure the diagnosis of PH. Our study evidenced a 40% prevalence of patients with elevated TRV at echocardiography. RHC (performed in 25 out of 32 patients) confirmed PH in 10% (95% CI 3.4-16.5%) of all patients, with a prevalence of post-capillary PH of 6.25% (95% CI 0.95-11.55%) and pre-capillary PH of 3.75% (95% CI -0.4-7.9%). Patients with PH were older, had worse performance in 6MWTs, and more pronounced anaemia, haemolysis and renal dysfunction. Survival was shorter in patients with PH.Our study reinforced the use of echocardiography as a screening tool for PH in SCD and the mandatory role of RHC for proper diagnosis. Our findings confirmed the prognostic significance of PH in SCD as its association to pronounced haemolytic profile.

show abstract

“…Haplotype 19/19 Haplotype 20/20 '# • yoshi et al [7] have suggested that a gene linked to the X chromosome and located at Xp22.2, influences the F cell production in both normal and SS patients; females with two X chromosomes are homozygous and may express higher levels of Hb F. Support for this possible link is pro vided by Nagel and Ranney [1] and Morris et al [8] who showed higher levels of Hb F in female SS patients, partic ularly for those older than 40 years. Gupta et al [5], how ever, did not observe such a difference between their male and female Indian SS patients.…”

mentioning

confidence: 76%

Level of Fetal Hemoglobin in Children with Sickle Cell Anemia Influence of Gender, Haplotype and α-Thalassemia-2 Trait

Adekile

Huisman²

1993

Acta Haematol

View full text Add to dashboard Cite

We have evaluated hematological data for numerous blood samples collected from 51 sickle cell anemia (SS) patients during their first 10 years of life. Haplo-types and α-globin gene status were determined in all patients. A total of 482 hematological evaluations and 317 fetal hemoglobin (Hb F) determinations are presented. The data show that the Hb F levels are the highest in patients with haplotypes 3/3 and the lowest in those with haplotypes 20/20. This is reflected in differences in total hemoglobin, PCV value, and RBC counts, which are the lowest in the 20/20 group and the highest in the 3/3 group. Female SS patients with haplotypes 19/19 have higher Hb F levels than their male counterparts; the same was observed for the patients with the 19/3 haplotype combination but not for those with the 20/3 haplotype combination. A concomitant α-thalassemia-2 heterozygosity had little or no effect on the hemoglobin level. The data confirm that SS children, aged 1-10 years, with a homozygosity for haplotype No. 20 are most severely affected by their disease.

show abstract

The haematology of homozygous sickle cell disease after the age of 40 years

Cited by 36 publications

References 11 publications

Sickle-Cell Disease in Brazzaville, Congo: Genetical, Hematological, Biochemical and Clinical Aspects

Sickle-Cell Disease in Brazzaville, Congo: Genetical, Hematological, Biochemical and Clinical Aspects

Pulmonary hypertension diagnosed by right heart catheterisation in sickle cell disease

Level of Fetal Hemoglobin in Children with Sickle Cell Anemia Influence of Gender, Haplotype and α-Thalassemia-2 Trait

Contact Info

Product

Resources

About