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Neutrophil elastase in patients with homozygous β‐thalassemia and pseudoxanthoma elasticum‐like syndrome
Author(s) -
Samarkos M.,
Aessopos A.,
Fragodimitri C.,
Karagiorga M.,
Kalotychou V.,
Voskaridou E.,
Kavouklis E.,
Loukopoulos D.
Publication year - 2000
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/(sici)1096-8652(200002)63:2<63::aid-ajh1>3.0.co;2-4
Subject(s) - pseudoxanthoma elasticum , elastase , medicine , pathogenesis , thalassemia , ceruloplasmin , neutrophil elastase , gastroenterology , endocrinology , pathology , chemistry , enzyme , biochemistry , inflammation
In this study we investigated the possible role of neutrophil (PMN) elastase and its natural inhibitor, α1‐proteinase inhibitor (α1‐PI) in the pathogenesis of the pseudoxanthoma elasticum (PXE)‐like syndrome which is found in patients with homozygous β‐thalassemia. We studied 30 β‐thalassemia homozygotes with the PXE‐like syndrome [PXE(+) group], 20 β‐thalassemia homozygotes without this syndrome [PXE(−) group] and 15 healthy controls. Plasma PMN elastase concentration in the PXE(+) and in the PXE(−) group was 136.4 ± 89 and 163.8 ± 126 μg/L, respectively ( P > 0.05). In the control group, the concentration was 42.9 ± 16.8 μg/L ( P < 0.01 for the comparison with both patients' groups). The plasma α1‐PI concentration in the PXE(+) and in the PXE(−) group was 2.28 ± 0.75 and 2.6 ± 0.96 g/L, respectively ( P > 0.05). Using logistic regression, we studied the prognostic value for PXE of the following independent variables: number of transfusions, chelation therapy, mean hemoglobin concentration, PMN elastase concentration, α1‐PI concentration, chronic transaminase elevation, and positivity for anti‐HCV. None of the above variables was found to have significant prognostic value for the PXE. Plasma PMN elastase concentration is elevated in all β‐thalassemia homozygotes; its role in the pathogenesis of the PXE‐like syndrome in β‐thalassemia can not be established, but our findings suggest that neutrophils of β‐thalassemia patients are activated, since PMN elastase is a marker of neutrophil activation. Am. J. Hematol. 63:63–67, 2000. © 2000 Wiley‐Liss, Inc.