Meet the Red Cell. K. Krishnan MD. FRCP, FACP. The red cell. Durability of red cell is remarkable No nucleus to direct regenerative processes No mitochondria available for efficient oxidative metabolism No ribosomes for regeneration of lost or damaged protein
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What is this?
What may have happened?
Rabbit spleen showing how RBC need to elliptically deform in order to pass through the very narrow slits in the wall of the splenic cords of Billroth and enter the sinusoids from which they can return to the circulation. A microspherocyte, deprived of its excess surface area, cannot ellipitically deform and is thus trapped in the cords.
Lower panel: Hereditary spherocytosis-lysis occurs in mildy hypotonic solutions
A model depicting the major proteins of the erythrocyte membrane is shown: α and β spectrin, ankyrin, band 3, 4.1 (protein 4.1), 4.2 (protein 4.2), actin, and GP (glycophorin).
How was it stained?
The Ham test (acidified serum lysis) establishes the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH), demonstrating a characteristic abnormality of PNH red blood cells by acidified fresh normal serum. Here is a PNH patient's (Pt) red blood cells lysed by normal serum at room temperature (RT) and at 37°C compared with normal red cells (no hemolysis) (control [C]). Heated serum at 56°C inactivates complement and prevents hemolysis in PNH cells.
(Taken from Image bank American Society of Hematology Slide Bank, 3rd edition.
Shortage of glycolipid molecule, GPI, due to a mutation in an X-linked gene called PIG-A
Somatic mutations and hence the patient’s marrow is a mosaic of PNH and normal cells