Splenic function in children with sickle cell disease: Two different patterns in Saudi Arabia

Babiker, M. A.; El‐Hazmi, Mohsen A. F.; Al‐Jobori, A.; Obeid, Hussein Al; Bahakim, Hassan M.

doi:10.1111/j.1600-0609.1985.tb01570.x

Cited by 14 publications

(3 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Adult patients with AI haplotype SCD bear some resemblance to patients with HbSC, given their higher haemoglobin levels, less haemolysis and a lower incidence of stroke and leg ulcers. Most children with AI haplotype SCD maintain near normal splenic function compared with African haplotype patients who commonly have early auto-splenectomy; this is probably related to the sustained high HbF in this population (Al-Awamy et al, 1984;Mallouh et al, 1984;Babiker et al, 1985;Al-Jam'a et al, 2000;Adekile, 2011;Alsultan et al, 2012b). Persistent splenomegaly and hypersplenism sometimes require splenectomy (Mallouh & Salamah, 1985;Al-Salem et al, 1996;Al-Salem, 2006).…”

Section: Discussionmentioning

confidence: 99%

“…Most children with AI haplotype SCD maintain near normal splenic function compared with African haplotype patients who commonly have early auto‐splenectomy; this is probably related to the sustained high HbF in this population (Al‐Awamy et al , ; Mallouh et al , ; Babiker et al , ; Al‐Jam'a et al , ; Adekile, ; Alsultan et al , ). Persistent splenomegaly and hypersplenism sometimes require splenectomy (Mallouh & Salamah, ; Al‐Salem et al , ; Al‐Salem, ).…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Sickle cell disease in Saudi Arabia: the phenotype in adults with the Arab‐Indian haplotype is not benign

et al. 2013

View full text Add to dashboard Cite

Summary Sickle cell disease (SCD) in Saudi patients from the Eastern Province is associated with the Arab-Indian (AI) HBB (β-globin gene) haplotype. The phenotype of AI SCD in children was described as benign and was attributed to their high fetal haemoglobin (HbF). We conducted a hospital-based study to assess the pattern of SCD complications in adults. A total of 104 patients with average age of 27 years were enrolled. Ninety-six percent of these patients reported history of painful crisis; 47% had at least one episode of acute chest syndrome, however, only 15% had two or more episodes; symptomatic osteonecrosis was reported in 18%; priapism in 17%; overt stroke in 6%; none had leg ulcers. The majority of patients had persistent splenomegaly and 66% had gallstones. Half of the patients co-inherited α-thalassaemia and about one third had glucose-6-phosphate dehydrogenase deficiency. Higher HbF correlated with higher rate of splenic sequestration but not with other phenotypes. The phenotype of adult patients with AI SCD is not benign despite their relatively high HbF level. This is probably due to the continued decline in HbF level in adults and the heterocellular and variable distribution of HbF amongst F-cells.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Sickle cell disease in Saudi Arabia: the phenotype in adults with the Arab‐Indian haplotype is not benign

et al. 2013

View full text Add to dashboard Cite

show abstract

“…Some particular SCD have equally been suspected for the maintenance of splenomegaly as they are associated with higher levels of HbF>20%, which reduces recurrent infarctions, normally responsible for autosplenectomy and eventually spleen atrophy [13,14,15]. Therefore the presence of these SCD does not lead to splenic atrophy but rather sustained splenic enlargement.…”

Section: Hemoglobin Fmentioning

confidence: 99%

Persistent Spleen Enlargement in Sickle Cell Disease: An Unresolved Dilemma

2020

ARC Journal of Pediatrics

View full text Add to dashboard Cite

Sickle cell disease (SCD) is a hereditary blood disorder which affects mostly blacks of African descent. Spleen enlargement or splenomegaly is a common clinical manifestation in SCD, and normally develops progressively from about the age of 6 months, and is due mainly to repeated capturing and hemolysis of sickled red blood cells. The spleen then undergoes atrophy by the age of 6 years, as a result of vasoocclusion, ischemia and scarring. However, in a few cases, this splenomegaly may persist beyond 6 years, and may be responsible for frequent hemolytic episodes and acute splenic sequestration of red blood cells which can be fatal. The cause, role, consequences and management of a persistent spleen enlargement remains unclear, and is still an unresolved dilemma. This review discusses these various aspects in light of recent research on this issue. From this we conclude that, there is a wide array of clinical implications and therapeutic options, which have to be considered in the management of these patients to avert deadly complications.

show abstract

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

et al. 1996

View full text Add to dashboard Cite

Spleen function was studied in a group of 20 Kuwaiti SS patients (aged 2-12 years), using 99mTc-labeled tin colloid scintigraphy. They were screened for the alpha-thalassemia determinants which are prevalent in the Arabian Peninsula [-alpha (3.7 kb) deletion, alpha2-globin gene polyadenylation signal (AATAAA => AATAAG) mutation, and 5' IVS-I splice junction pentanucleotide (GAGGTGAGG => GAGG) deletion] with a combination of polymerase chain reaction and allele-specific oligonucleotide (ASO) hybridization techniques. The patients were divided into three groups depending on the result of their colloid uptake. Group I consisted of 7 patients (35.0%) with normally visualized spleens, Group II consisted of 5 (25.0%) with partial visualization, and in Group III there were 8 (40.0%) in whom the spleen was not visualized at all. The significant distinguishing features among those in Groups I and III were mean corpuscular volumes (MCVs) of 74.1 +/- 5.1 and 90.1 +/- 6.6 fl (P<0.0001) and mean corpuscular hemoglobins (MCHs) of 22.4 +/- 2.7 and 27.5 +/- 4.0 pg (P<0.05), respectively. The overall frequency of alpha-thalassemia determinants in the study was 35.0%; however, the frequencies in Groups I, II, and III were 57.1, 30.0, and 18.8%, respectively. alpha-Thalassemia trait, therefore, appears to be associated with normal splenic function in these patients.

show abstract

Splenic function in children with sickle cell disease: Two different patterns in Saudi Arabia

Cited by 14 publications

References 18 publications

Sickle cell disease in Saudi Arabia: the phenotype in adults with the Arab‐Indian haplotype is not benign

Sickle cell disease in Saudi Arabia: the phenotype in adults with the Arab‐Indian haplotype is not benign

Persistent Spleen Enlargement in Sickle Cell Disease: An Unresolved Dilemma

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

Contact Info

Product

Resources

About