Despite the persistence of spherocytosis after splenectomy in hereditary spherocytosis, it has usually been assumed that red cell life span returns completely to normal after this treatment. Diisopropyl fluorophosphate. DF(32)P, a noneluting red cell label, was given intravenously to 11 patients in five unrelated families 2-27 yr after splenectomy for typical hereditary spherocytosis. Hemoglobi...

OBJECTIVE To determine whether patients with undiagnosed hereditary spherocytosis hospitalized for transfusions might have avoided hospitalization via earlier diagnosis. STUDY DESIGN Charts of all (N = 30) patients with hereditary spherocytosis seen in pediatric hematology at West Virginia University-Charleston were reviewed. Family and transfusion history and presence of neonatal jaundice we...

For many hereditary disorders, although the underlying genetic mutation may be known, the molecular mechanism leading to hemolytic anemia is still unclear and needs further investigation. Previous studies revealed an increased intracellular Ca2+ in red blood cells (RBCs) from patients with sickle cell disease, thalassemia, or Gardos channelopathy. Therefore we analyzed RBCs' Ca2+ content from 3...

OBJECTIVE To describe profile of 82 children with hereditary spherocytosis diagnosed over a period of 27 years (1985-2011) from a single center. METHODS Retrospective analyses of case records. RESULTS The mean (SD) age at diagnosis was 6.7 (2.8) years; 7 (8.5%) were diagnosed in infancy. Pallor (100%), icterus (67%), undocumented fever (28%), splenomegaly (96%) and hepatomegaly (73%) were t...

In contrast to the disease in humans, hereditary spherocytosis in the common house mouse produces an extreme spherocytosis. The cells show a broad distribution in size ranging from microcytic to macrocytic. Of particular interest is the finding of a substantial reduction in the major membrane polypeptide called spectrin, supporting a critical role for this protein in the control of erythrocyte ...

1. Cohen CM, Dotimas E, Korsgren C. Human erythrocyte membrane protein band 4.2 (Pallidin). Semin Hematol 1993; 30: 119-37. 2. Rybicki AC, Schwartz RS, Hustedt EJ, Cobb CE. Increased rotational mobility and extractability of band 3 from protein 4.2-deficient erythrocyte membranes: evidence of a role for protein 4.2 in strengthening the band 3-cytoskeleton linkage. Blood 1996; 88:2745-53. 3. Iol...

The use of glycosylated haemoglobin in the assessment of diabetic control is ubiquitous. Hereditary spherocytosis is a haemolytic anaemia with shortened red blood cell lifespan, which can interfere with the methods of glycosylated haemoglobin measurement. We report a case of hereditary spherocytosis in a young man with type 1 diabetes, and illustrate the discrepancy in the measurements of glyco...