Indications and Endpoints for Plasmapheresis in Sickle Cell Disease
Plasmapheresis (therapeutic plasma exchange/TPE) is NOT a standard first-line therapy for sickle cell disease complications—red cell exchange (RCE) remains the primary intervention—but TPE should be considered as an adjunctive rescue therapy specifically for acute multiorgan failure syndrome (MOFS) that remains refractory to red cell exchange. 1, 2
Primary Indication: Refractory Multiorgan Failure Syndrome
- TPE is indicated when patients with severe vaso-occlusive crisis develop progressive multiorgan failure despite adequate red cell exchange therapy. 1, 2
- The rationale is that plasma in SCD patients contains proinflammatory cytokines, adhesion molecules, and prothrombotic factors that promote further sickling and cause direct tissue toxicity beyond what RCE alone can address. 3
- In case series, TPE led to reversal of organ dysfunction with average time to initial laboratory improvement of 15.6 hours after first treatment. 1
- Survival rates approached 85% (6 of 7 patients) in critically ill patients with MOFS who received TPE after failing RCE, despite estimated mortality of 40%. 1, 2
Secondary Indications: Severe Acute Chest Syndrome and Intractable Pain
- TPE may be considered for severe acute chest syndrome when patients remain significantly hypoxic and in severe pain despite adequate red cell exchange. 3
- One documented case showed rapid clinical improvement and resolution of symptoms after adding TPE to RCE in refractory acute chest syndrome. 3
- TPE has shown benefit for intractable chest wall pain with impending acute chest syndrome, particularly in patients with history of hyperhemolysis where additional RCE carries risk. 3
- Rapid resolution of pain occurred irrespective of primary indication in multiple cases. 3
Clinical Endpoints for TPE Treatment
Discontinuation criteria include:
- Resolution of multiorgan dysfunction with stabilization of hemodynamics 1
- Laboratory evidence of decreased hemolysis (declining LDH, bilirubin, improved reticulocyte count) 1
- Clinical improvement in respiratory status and pain control 3
- Average ICU length of stay was 5.6 days and total hospital stay 14 days in survivors. 1
Technical Specifications
- Fresh frozen plasma should be used as replacement fluid. 2
- TPE was well tolerated with no adverse events reported in case series. 3
- TPE should be performed in addition to—not instead of—red cell exchange. 1, 2
Critical Distinction from Red Cell Exchange
It is essential to understand that standard sickle cell complications require red cell exchange, NOT plasmapheresis:
- Acute stroke requires emergency RCE with target HbS <30% (ideally <20%). 4
- Severe acute chest syndrome with respiratory failure requires RCE as first-line therapy. 4
- Sepsis with hemodynamic instability requires RCE. 4
- Preoperative preparation for high-risk surgery requires RCE. 5
The role of plasmapheresis is limited to the specific scenario of multiorgan failure that persists despite appropriate red cell exchange therapy. 1, 2