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(D58.0) Hereditary spherocytosis
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140 475 in individuals diagnosis hereditary spherocytosis confirmed
6 396 deaths with diagnosis hereditary spherocytosis
5% mortality rate associated with the disease hereditary spherocytosis
Diagnosis hereditary spherocytosis is diagnosed Men are 6.51% more likely than Women
74 807
Men receive the diagnosis hereditary spherocytosis
3 584 (4.8 %)Died from this diagnosis.
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65 668
Women receive the diagnosis hereditary spherocytosis
2 812 (4.3 %)Died from this diagnosis.
Risk Group for the Disease hereditary spherocytosis - Men aged 5-9 and Women aged 0-5
In Men diagnosis is most often set at age 0-94
Less common in men the disease occurs at Age 95+Less common in women the disease occurs at Age 95+
In Women diagnosis is most often set at age 0-94
Disease Features hereditary spherocytosis
Absence or low individual and public risk
Hereditary spherocytosis - what does this mean
Hereditary spherocytosis is an inherited disorder caused by a genetic defect that affects the membrane of red blood cells, resulting in them becoming rounder and more fragile than normal, leading to their premature destruction in the spleen. this causes a decrease in the number of red blood cells, which leads to anemia, jaundice, and other symptoms.
What happens during the disease - hereditary spherocytosis
Hereditary spherocytosis is a genetic disorder caused by mutations in the genes responsible for the production or assembly of red blood cell proteins, resulting in abnormal red blood cell shape and decreased red blood cell lifespan. this leads to anemia, jaundice, and splenomegaly as the body attempts to compensate for the decreased red blood cell lifespan. additionally, the abnormal red blood cell shape results in increased red blood cell destruction in the spleen, further contributing to the anemia.
Clinical Pattern
Hereditary spherocytosis is an inherited blood disorder characterized by the production of abnormally shaped red blood cells, known as spherocytes, which are unable to properly transport oxygen throughout the body. Symptoms can include anemia, jaundice, fatigue, and splenomegaly. Diagnosis is usually made through a combination of a complete blood count, reticulocyte count, and peripheral smear. Treatment typically involves splenectomy and/or blood transfusions.
How does a doctor diagnose
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Treatment and Medical Assistance
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8 Days of Hospitalization Required
Average Time for Outpatient Care Not Established
Hereditary spherocytosis - Prevention
Hereditary spherocytosis is a genetic disorder that affects red blood cells and can cause anemia. prevention of this condition involves genetic counseling and testing to identify the disorder in family members who may be at risk. additionally, maintaining a healthy lifestyle, including regular exercise and a balanced diet, can help prevent the onset of the condition.