Complementary markers for the clinical severity classification of hereditary spherocytosis in unsplenectomized patients.

摘要: Abstract Hereditary spherocytosis (HS) is usually classified as mild, moderate or severe using conventional features, namely, hemoglobin (Hb) concentration, reticulocyte count and bilirubin levels, which do not always contribute to an adequate clinical classification. The aim of our study was establish the importance some laboratory routine parameters, markers HS outcome, by studying a control group (n = 26) unsplenectomized patients (n = 82) presenting HS. We performed basic hematologic evaluated count, bilirubin, erythropoietin (EPO) soluble transferrin receptor (sTfR) levels; osmotic fragility (OFT) criohemolysis tests (CHT); ratios Hb/MCHC (mean cell concentration), Hb/RDW (red distribution width) MCHC/RDW, were calculated. Hb changed significantly in accordance with severity, but reticulocytes bilirubin. found that MCHC, RDW, EPO, sTfR, OFT, CHT calculated patients, and, therefore, valuable complementary diagnostic tools for Moreover, Hb/MCHC, MCHC/RDW worsening HS; thus, they are also good outcome In conclusion, we propose use these parameters useful complement analysis severity.