The Role Of The Spleen In Red Cell Membrane Disorders

The spleen plays a vital role in red cell health and longevity. Because 5% of cardiac output per minute is filtered through the spleen, this organ has ample opportunity to survey red blood cells for imperfections. Only those red cells that are deemed "flawless" are conducted through the rest of the red cell journey. The four functions of the spleen have been explained in Chapter 2, but when considering red cell membrane defects, it is the splenic filtration function that is the most relevant. As each red cell passes through the spleen, the cell is inspected for imperfections. Now imperfections may take many forms from inclusions to parasites to abnormal hemoglobin products or an abnormal membrane. Inclusions may be removed from the cell, leaving the membrane intact and allowing the red cells to pass through the rest of circulation unharmed. But if the red cell has abnormal hemoglobin (such as seen in thalassemia) or abnormal membrane components, then red cell elasticity and deformability are harmed. Some degree of hemolysis usually results. In the case of sphe-rocytes from hereditary spherocytosis, those red cells are less elastic and therefore the exterior membrane of the cell is shaved off, leaving a smaller, more compact red cell structure, a spherocyte. A spherocyte represents abnormal red cell morphology with a shortened life span and a low surface area to volume ratio (Fig. 7.1).

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