Neme-Yunes Y, Guerrero-Sánchez E, Pérez-Zepeda MU, López-Karpovitch X

Language: English
References: 23
Page: 233-238
PDF size: 288.79 Kb.

ABSTRACT

Background: Paroxysmal nocturnal hemoglobinuria (PNH), an acquired clonal bone marrow disorder due to somatic mutation of PIG-A gene, results in deficient expression of both glucosil-phosphatidylinositol (GPI) and proteins anchored to GPI on the surface of blood and hematopoietic cells. Despite frequent blood transfusion requirement, iron overload (IO) appears to be a rare condition in classic PNH.
Objetive: To determine the frequency of IO in patients with classic or hemolytic PNH.
Patients and Method: A study in which PNH diagnosis was established measuring by flow cytometry GPI-deficient cells in blood samples of patients with intravascular hemolytic anemia. IO was established as follows: in men, ferritin and transferrin saturation (TS) values ›300 ng/mL and ›50%, respectively. In women cut-off values for ferritin and TS were ›200 ng/mL and ›45%, respectively. Along with ferritin, ultrasensitive C-reactive protein (uCRP) was quantitated to rule out concomitant inflammation in all cases. A multiple linear regression model was employed to study the association between ferritin with TS, number of packed red blood cells (PRBC) units transfused and uCRP.
Results: 20 patients with classic PNH, 45% were women, median age 37 years were included. IO was identified in 8 cases (40%). A statistically significant correlation between ferritin with TS and ferritin with number of PRBC units transfused was recorded. In the multivariate analysis, the number of PRBC units transfused showed a highly statistical significant correlation with ferritin (p‹0.0001).
Conclusion: The association between increased ferritin values with PRBC transfusions must alert about the possibility of IO and its consequences in patients with classic PNH.

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