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Red cell ektacytometry in two patients with chronic hemolytic anemia and three new α-spectrin variants.
Ann Hematol
March 2022
Hematology Department, Hospital Universitari de La Santa Creu i Sant Pau, Barcelona, Spain.
Red blood cell (RBC) morphology is, in general, the key diagnostic feature for hereditary spherocytosis (HS) and hereditary elliptocytosis (HE). However, in hereditary pyropoikilocytosis (HPP), the severe clinical form of HE, the morphological diagnosis is difficult due to the presence of a RBC morphological picture characterized by a mixture of elliptocytes, spherocytes, tear-drop cells, and fragmented cells. This difficulty increases in new-borns and/or patients requiring frequent transfusions, making impossible the prediction of the disease course or its severity.
View Article and Find Full Text PDFEvaluation of red cell membrane cytoskeletal disorders using a flow cytometric method in South iran.
Turk J Haematol
March 2014
Diagnostic Laboratory, Sciences and Research Technology Center, Shiraz University of Medical Sciences, Shiraz, Iran ; School of Para Medical Sciences, Shiraz University of Medical Sciences, Shiraz, Iran.
Objective: The diagnosis of hereditary red blood cell (RBC) membrane disorders, and in particular hereditary spherocytosis (HS) and Southeast Asian ovalocytosis (SAO), is based on clinical history, RBC morphology, and other conventional tests such as osmotic fragility. However, there are some milder cases of these disorders that are difficult to diagnose. The application of eosin-5'-maleimide (EMA) was evaluated for screening of RBC membrane defects along with some other anemias.
View Article and Find Full Text PDFCharacterization of 13 novel band 3 gene defects in hereditary spherocytosis with band 3 deficiency.
Blood
December 1996
Department of Biomedical Research, St Elizabeth's Medical Center, Tufts University School of Medicine, Boston, MA, USA.
Hereditary spherocytosis (HS) is a common hemolytic anemia of variable clinical expression. Pathogenesis of HS has been associated with defects of several red cell membrane proteins including erythroid band 3. We have studied erythrocyte membrane proteins in 166 families with autosomal dominant HS.
View Article and Find Full Text PDF[Abnormal changes in erythrocyte membrane proteins in hereditary spherocytosis and their relation to clinical and biological aspects of the disease].
Med Clin (Barc)
June 1995
Servei d'Hematologia Biològica, Escola Professional d'Hematologia Farreras Valentí, Hospital Clínic i Provincial, Universitat de Barcelona.
Background: In the present paper we report a study of 20 patients with hereditary spherocytosis (HS) performed with the aim of provide further information on the electrophoretic abnormalities of red blood cell (RBC) membrane proteins and their putative relationship with the clinical, biological and genetic aspects of the disease.
Methods: General hematological parameters, reticulocyte count, osmotic fragility test and erythrocyte morphology analysis, were performed by routine procedures. Membrane proteins of erythrocyte were analyzed by SDS-polyacrylamide gradient gel electrophoresis (SDS-PAGE) using the Laemmli and Fairbanks methods.
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