La Hemolysis as a Predictor of Painful Crises in Sickle Cell Anemia

Vollständiger Abstract

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Background: Sickle cell anemia (SCA) is a genetic hematologic disorder resulting from homozygous inheritance of hemoglobin S (Hb S). It is characterized by chronic hemolytic anemia and complex manifestations. The altered Hb S molecule polymerizes under certain conditions, leading to red blood cell (RBC) sickling, hypoxia, vaso occlusion, and hemolysis. Intravascular hemolysis (IH) is considered a risk factor for severe conditions in SCA due to the release of cell-free plasma Hb and its toxic effects. This study aimed to evaluate these markers as potential risk factors for hemolysis and the occurrence of painful crises in SCA patients. Methods: A total of 103 SCA patients in steady state were included. Hematological and hemolytic parameters were analyzed, and plasma cell-free Hb levels were quantified. Patients were stratified into two groups based on the frequency of painful crises in the past year (0–2 crises and ≥3 crises). Results: Statistical analysis revealed that plasma Hb levels were significantly higher in the ≥3 crises group. Reticulocyte count, LDH, and AST levels correlated positively with plasma Hb levels, with reticulocyte count showing the highest influence. In conclusion, this study suggests that IH, as reflected by plasma Hb levels, may be a crucial hemolytic factor contributing to painful crises in SCA. Laboratory hemolytic markers such as LDH, AST, and reticulocyte count could aid in characterizing the hemolytic profile and predicting the severity of clinical manifestations, particularly vaso occlusive events and painful crises in SCA patients. Further research on genetic factors in SCA may provide additional insights into pain mechanisms and severity prediction.