Red cell calcium leak in congenital hemolytic anemia with extreme microcytosis

Red cell calcium leak in congenital hemolytic anemia with extreme microcytosis.

A child with congenital hemolytic anemia, extreme microcytosis and bizarre red cell morphology has been studied. Splenectomy at the age of 21 mo greatly improved the hemolytic anemia, although red cell morphology was unchanged. Aniso- and poikilocytosis were marked on a stained smear, and there were many small hyperchromatic cells of irregular shape. The MCV of 25 cu mu was very low and the MCH...

Red Cell Enzymopathies in Patients with Hemolytic Anemia in Southern Iran: Case Series

Abstract Background: Hereditary red cell enzyme disorders are a group of Non-immune/Spherocytic Hemolytic Anemia, although these disorders are rare and they have not public health problems, the detection of these defects could help to physician in treatment and differential diagnosis. This study evaluated 5 enzymopathies in patients with Hereditary Non –immune/Spherocytic Hemolyti...

Nonspherocytic congenital hemolytic anemia.

Altered sulfhydryl reactivity of hemoglobins and red blood cell membranes in congenital Heinz body hemolytic anemia.

The mechanisms of hemoglobin precipitation into Heinz bodies and hemolytic anemia that characterize congenital Heinz body hemolytic anemia (CHBHA) were studied in patients with the unstable hemoglobins, Köln (beta-98 valine --> methionine) and Hammersmith (beta-42 phenylalanine --> serine). The cysteines in the 93rd position of the beta-chains of CHBHA hemoglobins bound glutathione excessively ...

Acquired and Congenital Hemolytic Anemia.

1. Pediatricians and other general practitioners may not be aware of the significance of central nervous system disease in children who have sickle cell disease, particularly the more subtle silent infarct. 2. Pediatricians frequently fail to order a reticulocyte count or detect splenomegaly on physical examination before referring a patient with anemia. These findings are critical to diagnosin...