Iron-deficient state of patients with a clone of paroxysmal nocturnal hemoglobinuria

Karnabeda O.1, Huk N.2

Summary. As part of the analysis of the prevalence of iron deficiency in patients with paroxysmal nocturnal hemoglobinuria (PNH), a descriptive comparative study was conducted among 10 patients with PNH and the results of their general clinical and biochemical blood tests. Objective. Studying the prevalence of iron deficiency in patients with PNH and to determine a comprehensive approach to the treatment of anemia for patients with PNH. Materials and methods. For the study, 10 patients with a diagnosis of PNH and an existing PNH clone established by peripheral blood flow cytometry were selected. We analyzed the results of complete blood count, biochemical blood count, blood test for ferritin, transferrin, and transferrin iron saturation to determine the presence of iron deficiency or other causes of patients anemia. The available data were analyzed by descriptive research and comparison. Results. It was determined that 60% of patients with PNH have iron deficiency, and only 20% have functional iron deficiency. The mechanisms of iron deficiency in patients with PNH are still being studied. However, the approach to treatment in such patients should be comprehensively focused on rapid parenteral restoration of serum iron and prevention of the development of deficiency anemia. Conclusions. Iron deficiency for patients with PNH is most often caused by urinary iron loss due to chronic intravascular hemolysis (hemosiderinuria). As known, hemosiderinuria results the loss of up to 20 mg of iron per 24 hours, which leads to a negative iron balance and rapid depletion of iron stores. This requires a comprehensive approach to the treatment of iron deficiency in such patients.

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