Therapeutic plasma exchange in the management of acute complications of sickle cell disease: A single centre experience

Abstract

Sickle cell disease results in systemic inflammation even at steady state and this is accentuated during acute crises. The plasma of affected patients contains several proinflammatory cytokines as well as adhesion molecules and prothrombotic factors. This environment promotes further red cell sickling while many of these substances can cause direct tissue toxicity and end-organ damage. Even though red cell transfusion, whether simple or exchange, is the mainstay of treatment of severe acute complications, addition of therapeutic plasma exchange could potentially provide additional benefit by removing such harmful substances. Here, we describe two cases where therapeutic plasma exchange was used. The first involved a patient with the acute chest syndrome who despite adequate red cell exchange remained significantly hypoxic and in severe pain. We therefore proceeded to perform plasma exchange; this led to rapid clinical improvement and resolution of his symptoms. The second case involved a patient with intractable chest wall pain and impending acute chest syndrome; this patient also had a past history of hyperhaemolysis. The patient underwent therapeutic plasma exchange with very rapid resolution of the pain, avoidance of any respiratory deterioration and full recovery. We also give a brief summary of our previous experience using plasma exchange in patients with sickle cell disease. Plasma exchange was well tolerated with no adverse events in all cases we have treated, led to rapid resolution of pain irrespective of primary indication and in the majority of cases to a favourable clinical outcome.

Keywords: Red cell exchange; Sickle cell disease; Therapeutic plasma exchange.