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In fact, malaria is more serious in these patients. • The precise mechanism for this phenomenon in people with sickle cell trait is not known but several factors are believed to be responsible. – Infected red blood cells tend to have lower oxygen tension, because it is significantly reduced by the parasite. This causes sickling of that particular red blood cell and phagocytosis to get rid of the cell and hence the parasite within. – Since the sickling of parasite-infected cells is higher, these selectively get removed by the reticuloendothelial system, thus sparing the normal erythrocytes.

Presentations of Sickle Cell Anemia During Infancy • • • • • Anemia Repeated infections Failure to thrive Dactylitis (hand-foot syndrome) Splenomegaly • After recurrent episodes of sickling, membrane damage occurs and the cells are no longer capable of resuming the biconcave shape upon reoxygenation. Thus, they become irreversibly sickled cells (ISCs). From 5 to 50 % of RBCs permanently remain in the sickled shape. • When RBCs sickle (Figs. 23): – They gain Na+ and lose K+. – The red blood cells membrane permeability to Ca++ increases, possibly due, in part, to impairment in the Ca++ pump that depends on adenosine triphosphatase (ATPase).

In 1984, bone marrow transplantation in a child with sickle cell anemia produced the first reported cure of the disease. The transplantation was done to treat acute leukemia. The child’s sickle cell disease was cured as a side event. The procedure nonetheless set the precedence for later transplantation efforts directed specifically at sickle cell disease. Benefits of Hydroxyurea Treatment for Patients with Sickle Cell Anemia • • • • • It decreases the number and severity of vaso-occlusive attacks.

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