Anaemic crisis in sickle cell disease.

Area of Science:

• Hematology
• Pediatrics
• Immunology

Background:

• Sickle cell anemia (SCA) is a genetic blood disorder characterized by abnormal hemoglobin.
• Patients with SCA are prone to various complications, including acute anemia crises.

Purpose of the Study:

• To investigate the causes of acute anemia necessitating urgent blood transfusions in children with SCA.
• To identify potential triggers and underlying mechanisms of these severe anemia episodes.

Main Methods:

• Retrospective analysis of 16 episodes of acute anemia in 13 children with SCA.
• Evaluation of hemolysis markers, reticulocyte counts, and presence of cold agglutinins.
• Assessment for folate deficiency, G6PD deficiency, and splenic sequestration.

Main Results:

• Increased hemolysis was observed in 5/7 episodes; marrow erythroid failure in 10/16 episodes.
• Cold agglutinins with anti-I specificity were detected in most episodes (13/16 and 11/16 respectively).
• Infectious agents were suspected as a cause, ruling out folate deficiency, G6PD deficiency, or splenic sequestration.

Conclusions:

• Acute anemia crises in children with SCA can be triggered by cold agglutinins and possibly infectious agents.
• Warmed homologous blood transfusions are effective and safe, even with cross-matching difficulties.
• Understanding these triggers is crucial for managing SCA patients and preventing severe anemia episodes.