Lack of influence of fetal hemoglobin levels or erythrocyte indices on the severity of sickle cell anemia.

Powars, Darleen R.; Schroeder, W. A.; Weiss, Jie W; Chan, Linda S.; Azen, Stanley P.

doi:10.1172/jci109720

Cited by 99 publications

(39 citation statements)

References 34 publications

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“…This difference in symptomatology between the two groups was significant (P < 0.001; chi-square test). Further examination of the data showed that among 24 minimally symptomatic patients, only two had HbF of less than 18%, whereas among the 57 severely symptomatic patients, 14 had HbF values of less than 18%, a difference that was also significant (P < 0.05; chi-square test). Also, the mean HbF of asymptomatic patients (34.3% ± 12.6) was significantly higher than that of symptomatic patients (24.5% ± 11.7) (Table 3).…”

Section: Resultsmentioning

confidence: 92%

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Regional Variations in Sickle Cell Anemia in Saudi Arabia

et al. 1988

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We examined mean corpuscular volume (MCV), fetal hemoglobin (HbF) levels, and severity of clinical disease in 81 patients with sickle cell anemia from various regions of Saudi Arabia. We found that patients from eastern Saudi Arabia had less severe symptoms, higher mean Hb levels, and lower MCV than patients from other regions. Using a severity index, calculated as the mean value of individual symptoms and physical signs in patients in each region, we found that less severe symptoms were associated with lower MCV. High HbF levels were not confined to Eastern Province patients but were seen in other regions. There was a statistically significant inverse correlation between HbF% and the severity index. We also found that only two of the 24 minimally symptomatic patients had an HbF level less than 18%, whereas among the 57 severely symptomatic patients, 14 (25%) had an HbF level less than 18%, confirming the protective value of a certain minimum level of HbF.

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Section: Resultsmentioning

confidence: 92%

“…1,2,[11][12][13][14] In the present study, we evaluated clinical severity by determining the presence or absence of a number of symptoms and physical findings. These included bone pain, joint pain, anemia, jaundice, splenomegaly, osteomyelitis, aseptic necrosis of bone, and hand-foot syndrome.…”

Section: Clinical Severitymentioning

confidence: 99%

Regional Variations in Sickle Cell Anemia in Saudi Arabia

et al. 1988

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“…The frequency of painful crises in subjects of African origin appears unaffected by HbF level, and an analysis of hematological risk factors in painful crises failed to show any association with the HbF level. 40,43 Hematologically, HbF is consistently negatively correlated with HbA 2 and correlates positively with total hemoglobin and mean corpuscular volume in most age and sex groups. 44 Do these relationships also prevail in the Eastern Province or is the general level of HbF so high that there is insufficient variability to allow documentation of these effects?…”

Section: Questions On the Effects Of High Hbf Levelsmentioning

confidence: 97%

“…Studies on SS disease of African origin suggest that high levels of HbF are associated with lower counts of irreversibly sickled cells, 35 greater persistence of splenomegaly, 35 less delay in epi-physeal fusion, 36 more normal habitus, 37 earlier menarche, 38 and later onset of complications in early childhood, 39 as well as lesser risk of stroke, 40 proliferative sickle retinopathy, 41 and avascular necrosis of the femoral head. 42 High levels of HbF, by inhibiting sickling and allowing splenomegaly to persist, may also be a risk factor for chronic hypersplenism (Emond, unpublished observations).…”

Section: Questions On the Effects Of High Hbf Levelsmentioning

confidence: 99%

The Challenge of Sickle Cell Disease in Saudi Arabia

Serjeant

1990

Ann Saudi Med

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“…(9,10). La distribución geográfica particular de los haplotipos africanos (Senegal, Bantú, Camerún, Benín) alrededor del mundo, se derivó de patrones migratorios ancestrales a través del desierto del Sahara hacia el continente americano (2,11); se cree que el haplotipo asiático tiene un origen distinto al africano y está localizado básicamente en el golfo Pérsico y la India (12).…”

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