From total blood exchange to erythrocytapheresis and back to treat complications of sickle cell disease

Abstract

Erythrocytapheresis is an important procedure in the management of certain complications of sickle cell disease, including acute stroke, stroke prevention, acute chest syndrome, and multiorgan failure. Erythrocytapheresis in sickle cell disease simply entails the removal of the patient's red blood cells containing the abnormal sickle hemoglobin and replacing them with normal red blood cells carrying normal hemoglobin. In these procedures, the patient's plasma is not exchanged but is returned to the patient. Several studies have demonstrated that the plasma of patients with sickle cell disease contains several components that increase blood viscosity and initiate or promote vaso-occlusion. These factors include increased levels of globulins, especially immunoglobulin G, acute-phase reactants, fibrinogen, coagulation factors, inflammatory mediators, and heme in the steady state and increase further during painful crises. This may explain why, in certain complications of sickle cell disease, such as acute chest syndrome, hepatic crisis, and priapism, erythrocytapheresis by itself may not be effective despite repetitive cycles of red blood cell exchange. The use of therapeutic plasma exchange in addition to erythrocytapheresis in these situations seems to be useful in resolving them more efficiently. The role of therapeutic plasma exchange in the management of certain complications of sickle cell disease needs further evaluation. This commentary addresses the role of therapeutic plasma exchange in the management of complications of sickle cell disease.

Evolving treatment paradigms in sickle cell disease

“…In the current setting of improvement in RBC phenotyping and iron chelation therapy, evaluation on chronic transfusion in sickle cell patients is warranted. [51][52][53]…”

Transfusion‐transmitted malaria masquerading as sickle cell crisis with multisystem organ failure

“…In addition, plasmapheresis to decrease plasma-free heme and other factors that increase blood viscosity or promote vasoocclusion should be considered in life-threatening cases of sickle cell crisis. 27 In this case, the patient's hemolysis stabilized following tandem erythrocytapheresis and plasmapheresis, which were initiated after the development of MSOF and inadvertently lowered the parasite load, suggesting a role for these interventions even before initiation of antimalarial medications. Although the patient did experience multiple complications including CVA, likely secondary to the underlying moyamoya disease, he did not succumb to infection, highlighting the importance of timely diagnosis and appropriate management of MSOF.…”

“…We also advocate that acute malaria be on the differential diagnosis of such patients and, when present, that they be treated aggressively with RBC exchange transfusion. In addition, plasmapheresis to decrease plasma‐free heme and other factors that increase blood viscosity or promote vaso‐occlusion should be considered in life‐threatening cases of sickle cell crisis . In this case, the patient's hemolysis stabilized following tandem erythrocytapheresis and plasmapheresis, which were initiated after the development of MSOF and inadvertently lowered the parasite load, suggesting a role for these interventions even before initiation of antimalarial medications.…”

Case series supporting heme detoxification via therapeutic plasma exchange in acute multiorgan failure syndrome resistant to red blood cell exchange in sickle cell disease

“…These results are consistent with Hp or Hx limiting the Hb nitric oxide scavenging, extravasation, and free heme release that perpetuates oxidative tissue damage, lending support for a carefully conceived randomized controlled trial of TPE in severely ill SCD patients failing or unable to undergo standard RCE (such as those with multisystem organ failure, hyperhemolysis syndrome, or no available RBCs due to alloimmunization) to more definitively establish causal linkage. Indeed, consensus is growing regarding the need to further examine the role of TPE in SCD …”