hereditary spherocytosis


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Related to hereditary spherocytosis: Hereditary elliptocytosis

hereditary spherocytosis

[hə′red·ə‚ter·ē ‚sfer·ō‚sī′tō·səs]
(medicine)
A chronic congenital disorder of the erythrocytopoietic system characterized by a preponderance of spherical erythrocytes, increased osmotic fragility, hemolytic anemia, and splenomegaly.
References in periodicals archive ?
Chasis, "Hereditary spherocytosis and hereditary elliptocytosis: aberrant protein sorting during erythroblast enucleation," Blood, vol.
Vercellati et al., "Clinical and hematologic features of 300 patients affected by hereditary spherocytosis grouped according to the type of the membrane protein defect," Haematologica, vol.
Parvovirus infection in a family associated with aplastic crisis in an affected sibling pair with hereditary spherocytosis. J clin pathol.
Indications for surgery Indication n ITP 13 HS 12 SCD 3 CHA 2 Malignancy 2 Trauma 1 HS = hereditary spherocytosis; ITP = idiopathic thrombocytopaenic purpura; CHA = congenital haemolytic anaemia; SCD = sickle cell disease.
Frequent de novo moncallelic expression of R-spectrin gene (SPTB) in children with hereditary spherocytosis and isolated spectrin deficiency.
Splenectomy may help eliminate the main site of red-blood-cell destruction, as previously reported in patients with hereditary spherocytosis.
The results are conflicting regarding the degree of hemolysis, when hereditary spherocytosis and heterozygous [beta] thalassemia coexist.
Currently common indications for splenectomise in children are hereditary spherocytosis, Idiopathic Thrombocytopenic Purpura intractable to medication [5] and others like Thalassemia with splenomegaly and metabolic disorders.
Human serum "parvovirus": a specific cause of aplastic crisis in children with hereditary spherocytosis. J Pediatr
Serological screening was repeated before vaccination in leukemic patients and during the last visit of patients with thalassemia major and hereditary spherocytosis.
Hereditary spherocytosis RBC spectrin RBC ankyrin Hereditary elliptocytosis RBC spectrin Hereditary pyropoikilocytosis RBC spectrin Hemolytic anemia - Glucose- G-6-PD 6-phosphate dehydrogenase deficiency Hereditary nonspherocytic PDH hemolytic anemia - Pyruvate kinase deficiency Porphyrias Various genes encoding enzymes of the heme biosynthesis pathway
In reviewing a slide from a patient with hereditary spherocytosis, participants were advised to go over our policy regarding elevated MCHC results.
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