General Information About Hereditary Spherocytosis
Disease Details and Frequently Asked Questions
It is a condition where red blood cells, which are normally flat and flexible (like a donut), become rigid, small spheres (spherocytes) due to a deficiency in the proteins that make up the cell's skeleton.
The spleen, the blood's filtration center, identifies these misshapen, inflexible spherical cells as damaged or foreign as they pass through its narrow channels. It rapidly destroys them (hemolysis), leading to anemia.
Due to the continuous destruction of blood cells, fatigue and paleness (anemia) are observed. In addition, there is jaundice in the eyes caused by bilirubin released from the destroyed cells, and spleen enlargement (splenomegaly) resulting from overwork.
The excessive amount of bilirubin (yellow pigment) constantly spilling into the blood from the destroyed blood cells accumulates and crystallizes in the gallbladder, causing black pigment stones to form at very early ages.
Sphere-shaped cells are seen in the 'Peripheral Smear' test, where blood is examined under a microscope. A definitive diagnosis is made using the 'Osmotic Fragility Test', which measures how easily these cells burst in saltwater.
Folic Acid vitamins are given for life to support the blood production process. The definitive solution for the disease (the destruction process) is the surgical removal of the spleen, which unfairly destroys the cells, usually performed after the child is 6 years old.
Our health library contents are prepared for informational purposes only and with scientific data available at the time of recording. For all your questions, concerns, diagnosis, or treatment regarding your health, please consult your doctor or a healthcare institution.
