Overt iron deficiency in sickle cell disease

Area of Science:

• Hematology
• Genetic Blood Disorders

Background:

• Sickle cell disease (SCD) is a group of inherited red blood cell disorders.
• Iron deficiency is a common comorbidity that can affect red blood cell parameters.

Observation:

• Four patients with sickle cell disease (three with sickle cell anemia (SS) and one with sickle cell hemoglobin C (SC) disease) presented with overt iron deficiency.
• Blood loss was identified as the likely cause of iron deficiency in these patients.

Findings:

• Iron repletion in these patients led to improvements in hemoglobin, hematocrit, mean corpuscular volume, and mean corpuscular hemoglobin concentration (MCHC).
• Red blood cell morphology shifted from hypochromic microcytic to normochromic normocytic following iron treatment.
• Diagnosis of iron deficiency was confirmed by low serum ferritin and/or high total iron-binding capacity.
• Two patients experienced an increase in painful crises after iron repletion, and one patient had new-onset crises.

Implications:

• The findings suggest a complex relationship between iron status and sickle cell disease complications.
• The potential benefit of a lowered MCHC in sickle cell disease patients warrants further investigation.
• Managing iron deficiency in SCD requires careful consideration of its impact on disease activity.