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Anemia, Hereditary Spherocytic Hemolytic

Abstract

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Synonyms of Anemia, Hereditary Spherocytic Hemolytic

Acholuric Jaundice
Chronic Acholuric Jaundice
Congenital Hemolytic Anemia
Congenital Hemolytic Jaundice
Congenital Spherocytic Anemia
Hereditary Spherocytosis
HS
Icterus (Chronic Familial)
Minkowski-Chauffard Syndrome
SPH2
Spherocytic Anemia
Spherocytosis

Disorder Subdivisions

No subdivisions found.

General Discussion

Hereditary spherocytic hemolytic anemia is a rare blood disorder characterized by defects within red blood cells (intracorpuscular) that result in a shortened survival time for these cells. Red blood cells (erythrocytes) normally circulate for a few months and when they die off is replaced by new erythrocytes. However, in hereditary spherocytic hemolytic anemia, the cells die prematurely. They also have low amounts of fats (lipid) in the cell membranes and an abnormally small amount of surface area. The red blood cells are sphere-shaped (spherocytic) making it difficult for them to pass through the spleen, resulting in the early destruction of these cells (hemolysis). The sphere shape of the red blood cells is the hallmark of this disorder, and this abnormality may be identified under a microscope. Hereditary spherocytic hemolytic anemia is caused by an inherited metabolic defect.

Organizations related to Anemia, Hereditary Spherocytic Hemolytic

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