Keywords
intravascular hemolysis
iron metabolism
eculizumab
ravulizumab
T2* MRI of liver and kidneys
How to Cite
Keywords
Abstract
BACKGROUND. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal hematologic disease manifesting among others in hemolytic anemia. Due to predominantly intravascular hemolysis, iron metabolism in PNH patients is characterized by a number of features. Pathogenetic treatment with complement C5-inhibitors modifies the course of hemolysis and results in iron metabolic changes.
AIM. To characterize the serum parameters of iron metabolism in PNH patients and to evaluate T2* MRIs of the liver and kidneys.
MATERIALS & METHODS. Serum parameters of iron metabolism measured in 97 analyses and 99 T2* MRIs of the liver and kidneys were assessed in 82 PNH patients treated at the National Research Center for Hematology from 2018 to 2024. Patients were 21–76 years of age (median 39 years); there were 38 women and 44 men.
RESULTS. The analysis focused on the data from 78 PNH patients. Among non-recipients of C5-inhibitors, iron overload in liver tissue was identified in 10/38 (26.3 %) patients. In C5-inhibitor recipients, iron overload in liver tissue was found significantly more often, i.e. in 28/40 (70 %) patients. Iron overload in liver tissue was more pronounced in patients with suboptimal hematologic response to PNH therapy. A renal T2* MRI was performed in 71 patients. In all non-recipients of C5-inhibitors (n = 34), iron overload in renal tissue was detected. In C5-inhibitor recipients, iron overload in renal tissue occurred significantly less often, i.e. in 21/37 (57 %) patients.
CONCLUSION. Iron overload in renal cortex was identified in all PNH patients who were not treated with C5-inhibitors. Iron overload in liver tissue was associated with blood transfusions and suboptimal hematologic response to pathogenetic PNH therapy. In evaluating iron overload in liver and renal tissues, laboratory methods for assessing iron metabolism in PNH patients have lower sensitivity compared to T2* MRI. In PNH patients, the achievement of optimal response to C5-inhibitor treatment is associated with a decrease in severity of iron overload in renal tissue and no hepatic hemosiderosis.
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