Influence of α-thalassemia trait on spleen function in sickle cell anemia patients with high HbF

Adekile, Adekunle; Tuli, Mahmoud; Haider, M.Z.; Al-Zaabi, K.; Mohannadi, S.; Owunwanne, Azu

doi:10.1002/(sici)1096-8652(199609)53:1<1::aid-ajh1>3.0.co;2-v

Cited by 40 publications

(9 citation statements)

References 20 publications

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“…Since Hb F is uniformly high in our patients, it is not surprising that no correlation was found with TAMV. This is similar to our previous studies on avascular necrosis of the femoral head and spleen function in which no association was found with Hb F [22,23].…”

Section: Discussionsupporting

confidence: 92%

Transcranial doppler and brain MRI in children with sickle cell disease and high hemoglobin F levels

Asbeutah

Gupta

Al-Saeid

et al. 2013

Pediatric Blood & Cancer

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Section: Discussionsupporting

confidence: 92%

Transcranial doppler and brain MRI in children with sickle cell disease and high hemoglobin F levels

Asbeutah

Gupta

Al-Saeid

et al. 2013

Pediatric Blood & Cancer

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“…The other ameliorating factor in our patients is the high prevalence of ␣-thal trait, which was found in about 50% of the cases in the present study. Previous studies from our center showed that SS patients with co-inherited ␣-thal trait were more likely to retain good spleen function, have a reduced incidence of infections [23] and reduced prevalence of gallstones probably because of a less severe chronic hemolysis [24] when compared to SS patients with low Hb F levels.…”

Section: Discussionmentioning

confidence: 82%

Tumor necrosis factor-α is undetectable in the plasma of SS patients with elevated Hb F

et al. 2000

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Steady-state sickle cell disease (SCD) patients may have increased plasma levels of acute phase reactants and pro-inflammatory cytokines because of subclinical inflammation. We have estimated TNF-levels in the plasma and in supernatants following peripheral blood mononuclear cell (PBMC) activation with phytohemagglutinin (PHA) in a group of Kuwaiti SCD patients using ELISA. The group consisted of 28 SS, 8 S-thal, and 2 SD patients all in steady state; 5 SS patients were studied during 7 episodes of painful crisis. The subjects were aged 2 to 16 years, with a mean of 7.3 ± 3.5 years. The S-globin gene cluster haplotype,-tha1 status, and spleen function were determined in the SS group using standard techniques. Most (82%) were homozygous for the Saudi Arabia/ India haplotype and had elevated Hb F levels ranging from 15% to 35%. There were 24 controls (Hb AA or AS), of whom 14 were healthy and 10 were acutely ill at the time of the study. None of the children with SCD (either in steady state or crisis) had detectable plasma TNF-, but four controls (3 acutely ill and one healthy) had levels ranging from 61.7 to 249.8 pg/mL. Following PHA stimulation most subjects responded with high levels of TNF-, with the median level among the steady-state SS patients being significantly higher than that in the controls (both the acutely ill or healthy). It therefore appears that because of the mild disease among our Arab SS children, TNF-is not detectable in their plasma in steady state; these children, however, had a significantly higher response than controls following PBMC activation. Am. J. Hematol. 64:91-94, 2000.

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“…This contributes significantly to the amelioration of the disease and protects the patients from many of its complications [23]. Overt stroke, chronic leg ulcers, priapism, acute chest syndrome, and early splenic dysfunction are uncommon [16,24,25]. However, a subset of patients exists who have frequent, severe painful crises, many of whom end up with osteonecrosis.…”

Section: Fig 1 Mri Of Brain (T2-weighted) Axial Section Showing Hypmentioning

confidence: 99%

Silent brain infarcts in adult Kuwaiti sickle cell disease patients

et al. 2003

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Although overt stroke is a common complication of sickle cell disease (SCD), its incidence is very low in Kuwaiti patients. On the other hand, the prevalence of silent brain infarcts, which is reported to be about 17-20% in American patients, has not been documented in adult Kuwaiti patients. This is a 1-year study of consecutive, asymptomatic SCD patients seen in the hematology clinic of Mubarak Al-Kabeer Hospital. Patients with a past history of seizure or any other neurological abnormality were excluded. The patients' charts were reviewed for frequency of hospitalizations, any documented complications, and steady-state CBC. MRI was done with a 1.5-Tesla unit with super-conducting magnet. T1-and T2-weighted sagittal and axial sections and proton-density axial images were obtained in 5-mm-thick sections. Thirty-five patients were studied, made up of 25 SS and 10 S␤ 0 Thal, aged between 17 and 44 years, with a mean age of 26.9 ± 9.3 years. MRI findings consistent with infarcts were found in 7 (20.0%) patients-6 SS and 1 S␤ 0 thalwith a mean age of 31.8 ± 8.2 years, which was significantly higher (P < 0.05) than the mean age of the unaffected group (25.1 -9.0 years). There were also no differences in the mean Hb, Hb F, or any other hematological parameter in the two groups. Among the affected 6 SS, 2 had co-existent ␣-thal trait. It is interesting that, while silent infarcts are prevalent in young American patients, it is in the older age group that they occur in Kuwaiti patients. Further studies are needed to investigate the factors modulating this heterogeneity. Am. J. Hematol. 73:240-243, 2003.

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Influence of α-thalassemia trait on spleen function in sickle cell anemia patients with high HbF

Cited by 40 publications

References 20 publications

Transcranial doppler and brain MRI in children with sickle cell disease and high hemoglobin F levels

Transcranial doppler and brain MRI in children with sickle cell disease and high hemoglobin F levels

Tumor necrosis factor-α is undetectable in the plasma of SS patients with elevated Hb F

Silent brain infarcts in adult Kuwaiti sickle cell disease patients

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