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- Hereditary spherocytosis is a condition that affects red blood cells. People with this condition typically experience a shortage of red blood cells (anemia), yellowing of the eyes and skin (jaundice), and an enlarged spleen (splenomegaly).
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Jul 4, 2023 · Hereditary spherocytosis (HS) is the most prevalent cause of hemolytic anemia due to an abnormal red cell membrane and classifies as a type of congenital hemolytic anemia. Oskar Minkowsky first described it in the early 1900s.[1]
- Edgar A. Zamora, Catherine A. Schaefer
- 2023/07/04
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- What Is Hereditary spherocytosis?
- Who Is Affected by Hereditary spherocytosis?
- How Does Hereditary Spherocytosis Affect My body?
Hereditary spherocytosis is an inherited blood disorder that causes hemolytic anemia. This anemia happens when your red blood cellsbreak down faster than normal. In hereditary spherocytosis, your red blood cells lose their characteristic disk-like shape, becoming round or spherical cells (spherocytes) that are removed from circulation faster than n...
Hereditary spherocytosis typically affects people who have Northern Europe or North American ancestry. (Northern Europe is the northern part or region of the European continent.) Data vary on how many people have hereditary spherocytosis. Healthcare providers estimate that 1 person in 2,000 to 5,000 people worldwide may have hereditary spherocytosi...
In addition to developing hemolytic anemia, most people who have hereditary spherocytosis may have the following conditions: 1. Enlarged spleen (splenomegaly): People develop enlarged spleens in hereditary spherocytosis when their spleens fill up with spherocytes. Blood cells flow through your spleen, navigating several narrow passages before movin...
Oct 25, 2023 · The genetics, pathophysiology, clinical features, diagnosis, and treatment of HS will be reviewed here. Other inherited RBC membrane disorders, including hereditary elliptocytosis (HE), hereditary pyropoikilocytosis (HPP), and hereditary stomatocytosis (HSt), are discussed separately, as are general approaches to the evaluation of hemolytic anemia.
Feb 14, 2024 · Hereditary spherocytosis is a disorder in which the red blood cells are abnormal and fragile. Learn about the causes, symptoms, and treatment options for this disorder today.
Hereditary spherocytosis is a condition that affects red blood cells. People with this condition typically experience a shortage of red blood cells ( anemia ), yellowing of the eyes and skin (jaundice), and an enlarged spleen (splenomegaly).
Hereditary spherocytosis (HS) is a congenital hemolytic disorder wherein a genetic mutation coding for a structural membrane protein phenotype causes the red blood cells to be sphere-shaped (spherocytosis), rather than the normal biconcave disk shape.
2 days ago · The key laboratory features are the presence of spherocytes on the blood smear in association with a negative direct anti-globulin test (DAT) and an elevated reticulocyte count. May be newly diagnosed in children who present with severe anaemia due to parvovirus infection.
