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How to reach rapid diagnosis in sickle cell disease?
Ehsan Valavi1, Mohammad Javad Alemzadeh Ansari, Khodamorad Zandian
1Department of Nephrology, Abuzar Pediatric Hospital, Jundishapour University of Medical Sciences, Ahvaz, IR Iran.
Insights
Sickle cell disease (SCD) diagnosis in Iranian children is often delayed. Key indicators like low Hb×RBC product and high MCH/RBC ratio can help identify SCD in anemic patients with pain crises.
Area of Science:
- Hematology
- Pediatrics
- Genetics
Background:
- Sickle cell disease (SCD) is a prevalent hereditary condition in Iran.
- Newborn screening for SCD is standard in developed nations but often lacking in developing countries, leading to delayed diagnoses.
- This study examines the clinical and hematological features of SCD in Iranian children.
Purpose of the Study:
- To investigate the clinical presentation of sickle cell disease in Iranian pediatric patients.
- To compare hematological indices of SCD patients with those of healthy children.
- To identify specific hematological markers for early SCD detection.
Main Methods:
- The study included 44 pediatric patients diagnosed with sickle cell anemia (SS), sickle/β°-thalassemia (Sβ°), and sickle/β(+)-thalassemia (Sβ(+)).
- Fifty-seven healthy children were recruited as a control group.
- Hematological indices including Hemoglobin (Hb) concentration, Red Blood Cell (RBC) count, Hb×RBC product, Mean Corpuscular Volume (MCV), and Mean Corpuscular Hemoglobin (MCH) were analyzed.
Main Results:
- Patients with SS showed significantly lower Hb concentration, RBC count, and Hb×RBC product compared to controls (P<0.001).
- No significant differences in MCV and MCH were observed between SS patients and controls.
- An Hb×RBC product below 45 and an MCH/RBC ratio above 7 demonstrated high sensitivity and specificity (91-100%) for differentiating SCD from controls.
- The mean age at diagnosis was higher for Sβ(+) patients (7.45 years) compared to SS (4.3 years) and Sβ° (4.25 years) groups (P<0.001).
Conclusions:
- SCD should be suspected in anemic pediatric patients presenting with pain crises.
- Normochromic, normocytic anemia combined with an Hb×RBC product <45 and MCH/RBC ratio ≥7 are strong indicators for considering SCD.
- Prompt evaluation and confirmation of SCD are recommended based on these clinical and hematological findings.
Objective:
Sickle cell disease (SCD) is a common hereditary disease in Iran. In developed countries, newborn screening programs have been established to ensure early diagnosis, but in most developing countries, screening is not performed and the diagnosis is often delayed. The aim of the present work was to investigate the clinical presentation of SCD in Iran and comparison of its hematologic indices with normal children.
Methods:
The study included 44 pediatric patients (26 boys and 18 girls) with sickle cell anemia (SS), 27 sickle /β°-thalassemia (Sβ°), and 21 sickle /β(+)-thalassemia (Sβ(+)). Fifty seven healthy individuals matched with the patients were randomly selected as controls.
Findings:
Mean age at diagnosis in SS group was 4.3 years. At the time of diagnosis all patients were anemic, 89% complained of painful crises. Hemoglobin(Hb) concentration, red blood cell (RBC) count and Hb×RBC product in SS group was significantly lower than in control group (P<0.001), mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH) showed no significant differences. Hb×RBC product below 45 and MCH/RBC above 7 have the best sensitivity and specificity for differenting SS group and the control normal group (91 and 98% for Hb×RBC and 89 and 100% for MCH/RBC respectively). Mean age at diagnosis in Sβ(+) group was higher than in SS and Sβ° groups (7.45 year vs 4.26 and 4.25 year) (P<0.001). In addition, Sβ° and Sβ(+) groups had significantly lower MCV, MCH, and Hb×RBC indices compared with control group.
Conclusion:
We suggest that in an anemic patient with history of pain crises, normochrome normocytic anemia, Hb×RBC <45 and MCH/RBC ≥7, SCD should be considered and the patient evaluated accordingly to confirm the diagnosis.
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