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[The spleen in hereditary spherocytosis]
P Gregor1, A Hromec, J Jakubovský
1Ustav patologickej anatómie LJ UK Bratislava.
Abstract:
Hereditary spherocytosis is the most common inherent, autosomal dominant hemolytic anemia. Mild splenomegaly, venostasis and common decrease of while pulp is characteristic for hereditary spherocytosis. Cords are filled with spherocytes, sinuses can be empty or squeezed. Both sinuses and veins include ghost erythrocytes that lost haemoglobin. They are seen light-microscopically in differential interferent contrast. Macrophages are numerous, sinus lining cells are hypertrophic. Erythrophagocytosis is hardly seen by electron microscope. Not all red blood cells are spherocytes. Spherocytes are seen well in electronmicroscopy. We did not observed transition of erythrocytes through sinus walls. In certain circumstances lots of ferritin is seen both intra and extracytoplasmically. Iron accumulation in cords can result in their fibrosis.