Article Sections

Introduction

Sickle cell disease (SCD) ( Table 1) involves a group of inherited hemoglobinopathies characterized by the predominance of hemoglobin S (Hb S), a structural variant of hemoglobin, within red blood cells (RBCs).  Unlike normal hemoglobin, Hb S polymerizes under hypoxic conditions, forming rigid, sickle-shaped RBCs.  Patients have lifelong chronic hemolysis and vasoocclusion ( Figure 1) that can cause both acute (eg, ischemic pain) and chronic complications (eg, chronic kidney disease).  This article focuses on specific complications of SCD and their management.

Infection

  • Chronic vasoocclusion causes splenic infarctions that result in functional hyposplenism.  Loss of splenic function significantly increases the risk for serious bacterial infection.  Normally, circulating opsonized bacteria are recognized by splenic macrophages and are destroyed.  This process is particularly important for protection against encapsulated bacteria

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Figures

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Images

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Tables

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