Splenic function in sickle cell anemia patients in Qatif, Saudi Arabia

Al‐Jam'a, Ali H.; Al-Dabbous, Ibrahim A.; Chirala, Sundara Krishna; Al-Majid, Hussain; Al-Ali, Jameela

doi:10.1002/(sici)1096-8652(200002)63:2<68::aid-ajh2>3.0.co;2-n

Cited by 14 publications

(13 citation statements)

References 27 publications

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“…Nevertheless, patients differ from African Americans phenotypically and have fewer episodes of stroke, priapism, and leg ulcers and a higher prevalence of splenomegaly. 14,[70][71][72] This might be related to differences in HbF levels or coinheritance of ␣-thalassemia.…”

Section: Southwestern Provincementioning

confidence: 77%

Fetal hemoglobin in sickle cell anemia

Akinsheye

Alsultan

Solovieff

et al. 2011

Blood

427

354

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Fetal hemoglobin (HbF) is the major genetic modulator of the hematologic and clinical features of sickle cell disease, an effect mediated by its exclusion from the sickle hemoglobin polymer. Fetal hemoglobin genes are genetically regulated, and the level of HbF and its distribution among sickle erythrocytes is highly variable. Some patients with sickle cell disease have exceptionally high levels of HbF that are associated with the Senegal and Saudi-Indian haplotype of the HBB-like gene cluster; some patients with different haplotypes can have similarly high HbF. In these patients, high HbF is associated with generally milder but not asymptomatic disease. Studying these persons might provide additional insights into HbF gene regulation. HbF appears to benefit some complications of disease more than others. This might be related to the premature destruction of erythrocytes that do not contain HbF, even though the total HbF concentration is high. Recent insights into HbF regulation have spurred new efforts to induce high HbF levels in sickle cell disease beyond those achievable with the current limited repertory of HbF inducers.

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Section: Southwestern Provincementioning

confidence: 77%

Fetal hemoglobin in sickle cell anemia

Akinsheye

Alsultan

Solovieff

et al. 2011

Blood

427

354

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“…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%

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

et al. 2013

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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.

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“…Sickle cell anemia is one of the most common hereditary blood disorders in Saudi Arabia and it occurs in two different forms, one form in South West of the country is similar to the African type and the other type in the Eastern province is considered to be less severe [25]. In the Eastern province of Saudi Arabia SCA is common with sickle cell trait [26]. The current study showed that the patients who were affected with sickle cell anemia were from different Saudi Arabia areas; Eastern Saudi Arabia is the least number of affected patients (1.5%) followed by Asseer; affected patients constituting (16.4%) and from Gaizan representing (82.1%) of the total sample 67 (100%).…”

Section: Discussionmentioning

confidence: 66%

The Spleen and Sickle Cell Anemia: A Contrast Enhanced Computerized Tomography Based Study

Ahmed¹,

Ayad²

2016

OJRad

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The spleen is one of the most frequently affected organs in sickle cell anemia (SCA). This study aims to characterize the spleen in sickle cell anemia patients using contrast enhanced computerized tomography scanning (CECT). 67 patients with SCA from different Saudi Arabian areas were enrolled; ages are ranged from 10 months to 28 years old. The spleen was assessed with CT for abdominal pain and/or unexplained fever. The evaluation was done at different contrast enhancement scanning phases. The study showed that the least number of affected patients was from Eastern Saudi Arabia (1.5%) followed by Asseer (16.4%) then Gazan representing 82.1%. The most common type of SCA affected the Saudi children is Hemoglobin SS Disease (Hb SS) constituting 41 (61.2%). The spleen size, lymph nodes size, spleen Hounsfield (HU), splenic vein diameter and the correlation with the associated findings were evaluated for all of the patients. In children affected with SCA: 26 (38.8%) have splenomegaly, 18 (26.9%) have atrophied spleen and 2 (3.0%) are with very small tissue like structure. Lesions found in the spleen were abscess, infarction, cyst, and calcifications. At the spleen hilum region; dilated splenic vein, presence of multiple collaterals, and thrombus were also been detected. Significantly correlations were noticed between lesions type, child age, enlargement of spleen and splenic lymph nodes at P ≤ 0.033, P ≤ 0.010 and P ≤ 0.012 respectively and showed an evidence that the reduction of the HU and advanced age have significant relation with changing of the spleen size at P ≤ 0.004 and P ≤ 0.000 respectively. Spleen lesions' enhancement pattern is well emerged in both venous and delay phase and it was significantly related with the scanning phase at P ≤ 0.000 and with different types of SCA at P ≤ 0.037, and P ≤ 0.055 in venous and delay phase in respectively. CECT offers a number of morphological criteria that can be applied to differentiate hypodense lesions of the spleen in SCA. CT characterization criteria of hypodense splenic lesions are acknowledged to aid interpretation during evaluation of abdominal CT images of the spleen in symptomatic patients with sickle cell anemia.

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Splenic function in sickle cell anemia patients in Qatif, Saudi Arabia

Cited by 14 publications

References 27 publications

Fetal hemoglobin in sickle cell anemia

Fetal hemoglobin in sickle cell anemia

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

The Spleen and Sickle Cell Anemia: A Contrast Enhanced Computerized Tomography Based Study

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