Spherocytosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Symptoms
The spleen's hemolysis results directly in varying degrees of anemia and hyperbilirubinemia, which in turn result in symptoms of fatigue, pallor, and jaundice.
Acute cases can threaten hypoxemia through anemia and acute kernicterus through hyperbilirubinemia, particularly in newborns.
Chronic symptoms include anemia and splenomegaly, or enlargement of the spleen due to its increased activity. In some cases the spleen continues to change sizes. Yet in other cases the spleen just continues growing which puts the patient at a greater risk for rupture, which can cause death. Furthermore, the detritus of the broken-down blood cells--bilirubin--accumulates in the gallbladder, and can cause gallstones or "sludge" to develop. In chronic patients, an infection or other illness can cause an increase in the destruction of red blood cells, resulting in the appearance of acute symptoms, a hemolytic crisis.
Diagnosis
In peripheral blood smears, many of the red blood cells will appear abnormally small and will lack the central pallor--the lighter area in the middle of a RBC as seen under a microscope.
The CBC (cell blood count) laboratory values will show elevated MCHC.
The splenic cords are congested with red blood cells to be destroyed and macrophages of the spleen will show signs of actively destroying erythrocytes (erythrophagocytosis). This will result in an elevated bilirubin level.
The bone marrow in its role of manufacturing red blood cells will display hyperplasia, the increased activity of replacing RBCs. As a result, immature red blood cell-or reticulocyte-counts will appear elevated.
See also
- Hereditary spherocytosis
- Anemia
- Blood
- Blood diseases
- Red blood cells
- Hereditary diseases
External links
References
- Kumar, Vinay, Abul Abbas, and Nelson Fausto. "Robbins and Cotran Pathologic Basis of Disease, 7th edition (2004)."
- Schneider, Arthur S. and Philip A. Stanzo. "Board Review Series: Pathology, 2nd edition (2002)."
de:Kugelzellenanämie
it:Sferocitosi
nl:Congenitale sferocytose