TY - JOUR
T1 - Complementary markers for the clinical severity classification of hereditary spherocytosis in unsplenectomized patients
AU - Rocha, Susana
AU - Costa, Elísio
AU - Rocha-Pereira, Petronila
AU - Ferreira, Fátima
AU - Cleto, Esmeralda
AU - Barbot, José
AU - Quintanilha, Alexandre
AU - Belo, Luís
AU - Santos-Silva, Alice
N1 - Funding Information:
This study was supported by a PhD grant ( SFRH/BD/22442/2005 ) attributed to S. Rocha by Fundação para a Ciência e Tecnologia (FCT) and Fundo Social Europeu (FSE). For blood collection we thank the nurse team from “Hematologia Clínica do Hospital S. João”, “Serviço de Hematologia Laboratorial do Hospital Santo António” and laboratory technician Elisabete Machado from “Serviço de Hematologia do Hospital Maria Pia.”
PY - 2011/2/15
Y1 - 2011/2/15
N2 - 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 to establish the importance of some laboratory routine parameters, as markers of HS clinical outcome, by studying a control group (n = 26) and unsplenectomized HS patients (n = 82) presenting mild, moderate or severe HS. We performed a basic hematologic study and evaluated the reticulocyte count, bilirubin, erythropoietin (EPO) and soluble transferrin receptor (sTfR) levels; the osmotic fragility (OFT) and criohemolysis tests (CHT); the ratios Hb/MCHC (mean cell hemoglobin concentration), Hb/RDW (red cell distribution width) and MCHC/RDW, were calculated. Hb changed significantly in accordance with HS severity, but not reticulocytes or bilirubin. We found that MCHC, RDW, EPO, sTfR, OFT, CHT and the calculated ratios were significantly changed in patients, and, therefore, were valuable as complementary diagnostic tools for HS. Moreover, RDW, Hb/MCHC, Hb/RDW and MCHC/RDW changed significantly with worsening of HS; thus, they are also good markers for the clinical outcome of HS. In conclusion, we propose the use of these routine parameters as useful to complement the analysis of HS severity.
AB - 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 to establish the importance of some laboratory routine parameters, as markers of HS clinical outcome, by studying a control group (n = 26) and unsplenectomized HS patients (n = 82) presenting mild, moderate or severe HS. We performed a basic hematologic study and evaluated the reticulocyte count, bilirubin, erythropoietin (EPO) and soluble transferrin receptor (sTfR) levels; the osmotic fragility (OFT) and criohemolysis tests (CHT); the ratios Hb/MCHC (mean cell hemoglobin concentration), Hb/RDW (red cell distribution width) and MCHC/RDW, were calculated. Hb changed significantly in accordance with HS severity, but not reticulocytes or bilirubin. We found that MCHC, RDW, EPO, sTfR, OFT, CHT and the calculated ratios were significantly changed in patients, and, therefore, were valuable as complementary diagnostic tools for HS. Moreover, RDW, Hb/MCHC, Hb/RDW and MCHC/RDW changed significantly with worsening of HS; thus, they are also good markers for the clinical outcome of HS. In conclusion, we propose the use of these routine parameters as useful to complement the analysis of HS severity.
KW - Clinical outcome
KW - Hereditary spherocytosis
KW - Severity markers
UR - http://www.scopus.com/inward/record.url?scp=79151483575&partnerID=8YFLogxK
U2 - 10.1016/j.bcmd.2010.11.001
DO - 10.1016/j.bcmd.2010.11.001
M3 - Article
C2 - 21138793
AN - SCOPUS:79151483575
SN - 1079-9796
VL - 46
SP - 166
EP - 170
JO - Blood Cells, Molecules, and Diseases
JF - Blood Cells, Molecules, and Diseases
IS - 2
ER -