Iron deficiency in Yemeni patients with sickle-cell disease

Abstract

Despite the general view that patients with sickle-cell disease (SCD) have iron overload, there are reports of iron deficiency in a proportion of these patients. We studied Yemeni patients aged 1-30 years with homozygous SCD to determine their iron status using a set of 4 criteria (low serum iron, low transferrin saturation, high total iron binding capacity and low mean corpuscular volume for age). Of the 75 patients, 44 had never been transfused while 31 patients had received blood transfusions but not during the 3-month period prior to the study. Of the patients, 10 (13.3%) met the criteria for iron deficiency, 9 of whom were from the non-transfused patients (20.5%). The sensitivity and specificity were 40% and 98% respectively for reticulocyte count and 80% and 90% respectively for reticulocyte index. We recommend screening non-transfused SCD patients for iron deficiency. Anémie ferriprive chez des patients yéménites atteints de drépanocytose RÉSUMÉ En dépit de l'idée communément admise selon laquelle les patients atteints de drépanocytose souffrent d'une surcharge en fer, certains d'entre eux sont atteints d'une anémie ferriprive. Nous avons examiné des patients yéménites âgés de 12 mois à 30 ans atteints de drépanocytose homozygote afin d'établir leur bilan en fer à l'aide de quatre critères (taux de fer sérique faible, taux de saturation de la transferrine faible, capacité totale de fixation du fer élevée et volume globulaire moyen faible par rapport à l'âge). Sur 75 patients, 44 n'avaient jamais été transfusés, 31 avaient reçu des transfusions sanguines mais pas dans les trois mois précédents l'étude. Parmi les patients, 10 (13,3 %) ont reçu le diagnostic d'anémie ferriprive, dont 9 appartenaient au groupe de patients n'ayant jamais été transfusés (20,5 %). La sensibilité et la spécificité étaient de 40 % et 98 % respectivement pour la numération des réticulocytes et de 80 % et 90 % respectivement pour l'indice de production des réticulocytes. Nous recommandons le dépistage de l'anémie ferriprive chez les patients drépanocytaires n'ayant jamais été transfusés.

Iron status in sickle cell disorders

“…In addition, in our study, serum iron values in transfused patients were significantly higher than those in nontransfused patients, which was statistically significant (P < 0.0000001). This is well correlated with the study by Kassim et al [13] . In our study, 59.5% of blood transfused patients and 21.2% of nontransfused patients showed increased serum iron values.…”

“…Mean value of reticulocyte count in iron-deficient group was 1.64 and in noniron-deficient group was 6.60. This correlated well with the study done by Kassim et al [13] in which mean value of reticulocyte in iron-deficient group was 2.5 and in noniron-deficient group was 9.7. This low reticulocyte count is a sign of impaired erythropoiesis, which is known to be associated with many conditions including iron, folate, and vitamin B12 deficiency, and when MCV is low for age, the reduced reticulocyte count/index supports the diagnosis of iron deficiency.…”

“…Results of current study were close to the study conducted by Patra et al [11] and Okeahialam and Obi [12] who found iron deficiency in 6% and 7.76% cases, respectively. However, iron deficiency was diagnosed by Kassim et al [13] using similar criteria as ours in Yemini patients in 13.3% and by Vichinsky et al [8] in 16% of nontransfused patients. Mohanty et al [14] using the single criteria of ZPP/heme ratio (ZPP/heme) for diagnosis in Indian subjects reported iron deficiency in 67.7% of SCDs patients and 26.2% of sickle cell trait patients, which is much higher than all other studies.…”

Study of Iron Profile in Sickle Cell Patients

“…Patel et al [10] found that 4 In the same study, it was also found that 5 (11.62%) transfused patients and 14 (25%) non-transfused patients had decreased serum iron. Kassim et al [11] found that serum iron values were more in transfused patients as compared to non-transfused patients. The study done by Ikusemoro et al [12] showed a positive correlation between serum ferritin and number of units of blood transfused with a linear increase in serum ferritin levels seen in cumulative transfusions.…”

Low educational level of head of household, as a proxy for poverty, is associated with severe anaemia among children with sickle cell disease living in a low‐resource setting: evidence from the SPRING trial

“…25 Additionally, low MCV or microcytosis is one of the indicators of iron deficiency anaemia, which accounts for more than half (c 53%) of the causes of anaemia globally, with the highest burden among children between ages five and 14 years in resource-limited countries. 26,27 The incidence of iron deficiency anaemia among individuals with SCA in resource-limited settings is estimated to be around 10-11%, 28,29 particularly in the non-transfused cohort. The cross-sectional nature of this analysis did not allow a detailed analysis of the haemoglobin phenotype or determination of iron studies in our cohort.…”