hereditary spherocytosis

Measuring iron stores is therefore considered part of the diagnostic approach to hereditary spherocytosis.

The term "non hereditary spherocytosis" is occasionally used, albeit rarely.

The primary defect in hereditary spherocytosis is a deficiency of membrane surface area.

At this point, there exists no cure for the genetic defect that causes hereditary spherocytosis.

It almost always refers to hereditary spherocytosis.

Spherocytes are found in hereditary spherocytosis and autoimmune hemolytic anemia.

Mutations in erythrocytic ankyrin 1 have been associated in approximately half of all patients with hereditary spherocytosis.

Individuals with reduced or defective ankyrinR have a form of hemolytic anemia termed hereditary spherocytosis.

Chauffard is remembered for his work involving liver disease and his pathophysiological research of hereditary spherocytosis.

Check the slide for spherocytosis (e.g. in hereditary spherocytosis, among other causes)

In hereditary spherocytosis, erythrocytes fail to pass through and get phagocytosed, causing extravascular hemolysis.

Defects of red blood cell membrane production (as in hereditary spherocytosis and hereditary elliptocytosis)

Although the osmotic fragility test is widely considered the gold standard for diagnosing hereditary spherocytosis, it misses as many as 25% of cases.

These changes are also seen in non-hereditary spherocytosis, but they are typically more marked in hereditary spherocytosis.

In people with sickle-cell disease or other forms of chronic hemolytic anemia such as hereditary spherocytosis, infection can precipitate an aplastic crisis.

Castle and his team later characterized the red blood cell defects that are responsible for paroxysmal nocturnal hemoglobinuria and hereditary spherocytosis.

Risk factors for pigment stones include hemolytic anemias (such as sickle-cell disease and hereditary spherocytosis), cirrhosis, and biliary tract infections.

MCHC is elevated ("hyperchromic") in hereditary spherocytosis, sickle cell disease and homozygous hemoglobin C disease.

A number of genes have been linked to common hereditary elliptocytosis (many involve the same gene as forms of Hereditary spherocytosis, or HS):

They are most commonly found in immunologically-mediated hemolytic anemias and in hereditary spherocytosis, but the former would have a positive direct Coombs test and the latter would not.

Partial splenectomies are sometimes performed as a treatment for anemias due to an underlying inability for RBC deformation (hereditary spherocytosis and pyruvate kinase deficiency).

Disorders of the proteins in these membranes are associated with many disorders, such as hereditary spherocytosis, hereditary elliptocytosis, hereditary stomatocytosis, and paroxysmal nocturnal hemoglobinuria.

Hereditary spherocytosis is a hereditary defect that results in defects in the RBC cell membrane, causing the erythrocytes to be sequestered and destroyed by the spleen.

In a peripheral blood smear, the abnormally small red blood cells lacking the central pallor as seen in non-hereditary spherocytosis is typically more marked in hereditary spherocytosis.

The ESR is increased in pregnancy, inflammation, anemia or rheumatoid arthritis, and decreased in polycythemia, sickle cell anemia, hereditary spherocytosis, and congestive heart failure.