Table 1.
Indications for therapeutic plasma exchange (TPE) in the ICU: absolute (likely or less likely be used), relative, and rescue therapy
| Disease | Rationale | Replacement fluid | Adjunct therapeutic options | Strategya and Endpoints | Parameters to monitor | Additional comments |
|---|---|---|---|---|---|---|
| Absolute indications: disorders for which TPE is a recognized first-line treatment [2] | ||||||
| Acute inflammatory demyelinating polyradiculoneuropathy (Guillain-Barré syndrome) | Removal of antibodies | Albumin or plasma | IVIG | 1–1.5 TPV, 5–6 sessions over 10–14 days until clinical improvement | Clinical response | Consider TPE if failed to respond to IVIG and/or impending respiratory failure |
| Anti-glomerular basement membrane disease (Goodpasture syndrome) | Removal of pathogenic autoantibodies (including anti-GBM antibodies) | Albumin; plasma if bleeding | Corticosteroids, cyclophosphamide, rituximab | 1–1.5 TPV daily or on alternate days over 10–20 days until disease control |
Renal function Clinical response |
Anti-GBM antibodies may fall to undetectable levels within 2 weeks; TPE course should be ≥ 10–20 days and should continue until resolution of glomerular or pulmonary injury The presence or absence of antibody should not guide decisions to initiate or end TPE |
| Hyper-viscosity syndrome (in hyper-gammaglobulinemia, especially Waldenström macroglobulinemia) | Removal of paraproteins, thereby reducing the plasma viscosity | Albumin or Albumin/saline | Systemic chemotherapy or immunotherapy | 1–1.5 TPV daily until symptoms subside, most often 1–3 procedures |
Clinical response M component (mainly IgM levels) |
Symptoms are more reliable than concrete values of viscosity or immunoglobulins to guide therapy |
| Catastrophic antiphospholipid syndrome | Removal of antibodies (including antiphospholipid antibodies), cytokines, and complement factors; administration of coagulation factors | Plasma (± albumin) | Anticoagulation, corticosteroids, IVIG, rituximab or eculizumab | 1–1.5 TPV daily or alternate days; until clinical response | Clinical response | |
| Myasthenia gravis | Removal of autoantibodies (including antiacetylcholine receptor antibodies) and immunomodulation | Albumin | Cholinesterase inhibitors, corticosteroids, immunosuppression, IVIG, thymectomy, eculizumab | 1–1.5 TPV; 3–6 sessions over 10–14 days, until disease control | Clinical response | More effective if initiated during myasthenic crisis, especially with bulbar or severe generalized response; more effective than IVIG in patients with MuSK-Ab |
| N-Methyl-d-aspartate receptor antibody encephalitis | Removal of antibodies (including anti-neuronal autoantibodies) | Albumin |
High dose corticosteroids, IVIG, occasionally rituximab or cyclophosphamide Tumor resection (when tumor is present) |
1–1.5 TPV; 5–12 sessions over 1–3 weeks until clinical response | Clinical response | Check for ovarian tumors and other tumors (germ cell tumors, carcinoma, teratoma, lymphoma) |
| Thrombotic thrombocytopenic purpura | Administration of ADAMTS13 protease and removal of anti-ADAMTS13 autoantibodies | Plasma | Corticosteroids, rituximab, Caplacizumab (recombinant ADAMTS13?) |
Daily until platelet count > 150 × 10.9/L, LDH approaching normal and resolution of non-fixed neurologic symptoms then Continue for 2 more sessions then stop |
Platelet count, LDH, ADAMTS13 activity | Recovery of ADAMTS13 activity to > 10% within 7 days is associated with clinical response |
| Acute liver failurea |
Removal of albumin-bound and water-soluble toxins Replacement of plasma proteins including clotting factors Immunomodulation Reduction of proinflammatory response |
Plasma | Multiorgan support | High-volume TPE if possible (target 8–12 L); otherwise, 1–1.5 TPV daily until clinical improvement or transplantation |
Clinical response Supportive care as a bridge to liver transplantation |
Always consider TTP in the differential in specific scenarios (e.g., pregnancy and acute liver failure) Supportive care may improve nontransplant outcome Support care may stabilize while awaiting liver transplant |
| Relative indications: Disorders for which TPE is a recognized second-line treatment (alone or combined) | ||||||
| Thyroid storm (refractory) | Removal of autoantibodies, catecholamines, and cytokines | Plasma, albumin | Propylthiouracil, corticosteroids, ß-blockers, cholestyramine, organ support | Daily to every 3 days, until control of systemic response | Clinical response | Although a category II per 8th ASFA guidelines, TPE could be considered in refractory cases |
| ANCA-associated vasculitis with diffuse alveolar hemorrhage | Removal of autoantibodies and inflammatory mediators | Plasma | Corticosteroids, rituximab, cyclophosphamide | 1–1.5 TPV daily or every other day until disease control | Clinical response (resolution of pulmonary hemorrhage) |
PEXIVAS trial suggested no benefit on death or end stage kidney disease Now category II per recent ASFA update [73] |
| Acute disseminated encephalomyelitis | Removal of presumedly pathogenic autoantibodies | Albumin | Corticosteroids, IVIG | 1–1.5 TPV every other day until disease control | Clinical response | |
| Thrombotic microangiopathy-complement-mediated (formerly known as atypical hemolytic syndrome (aHUS)) | Recommended while investigations for TTP and other forms of TMA are in progress or if eculizumab is not available | Plasma | Eculizumab | 1–1.5 TPV daily until TTP ruled out | Platelet count | |
| Autoimmune hemolytic anemia | Removal of pathogenic immune complexes, autoantibodies and complement components | Albumin | Corticosteroids, rituximab, IVIG, immunosuppression, monoclonal antibody therapy, splenectomy | TPV 1–1.5 daily until disease control | Clinical response | |
| Chronic acquired demyelinating polyneuropathies (IgA- and IgG-associated polyneuropathy) | Removal of autoantibodies | Albumin | IVIG and rituximab | 5–6 treatments over 10–14 days until improvement or stabilization of neurological response |
Clinical response Nerve conduction studies; IgG and IgM titers |
Frequency: 2–3/week until improvement, then tapered, e.g., weekly, or monthly |
| Lambert–Eaton myasthenic syndrome | Removal of autoantibodies | Albumin | Aminopyridines, possibly cholinesterase inhibitors; immunosuppression if symptomatic treatment is insufficient | 1–1.5 TPV daily or on alternate days until clinical response | Clinical response | |
| Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) or Hashimoto’s encephalopathy | Removal of autoantibodies | Albumin | Corticosteroids, IVIG, azathioprine, cyclophosphamide, potentially monoclonal antibody therapy |
1–1.5 TPV daily or on alternate days; 3–9 procedures until Clinical response |
Clinical response | Utilized in patients who have failed to respond to first-line therapy with corticosteroids |
| Rescue indications: disorders for which TPE may be used in the ICU as rescue therapy despite lack of strong evidence about efficacy | ||||||
| HIT with progressive thrombosis | Removal of platelet-activating HIT antibodies | albumin, plasma | Non-heparin anticoagulation | 1–1.5 TPV daily or on alternate days until clinical response | Clinical response; HIT antibody levels | |
| Cryoglobulinemia vasculitis | Removal of cryoglobulins | Albumin | Corticosteroids, cyclophosphamide, rituximab | 1–1.5 TPV every 1–3 days; 3–8 sessions until disease control | Clinical response | |
| Pancreatitis with severe hypertriglyceridemia | Decrease of triglyceride levels, removal of inflammatory cytokines, and potential replacement of deficient lipoprotein lipase | Albumin, plasma | Dietary restriction, lipid-lowering drugs, insulin, heparin | TPV 1–1.5 daily for 1–3 days until clinical response and triglyceride levels | Clinical response; triglyceride levels | |
| Paraneoplastic neurological syndromes | Removal of autoantibodies | Albumin | Antitumor therapy, immunosuppression (corticosteroids, IVIG) | 1–1.5 TPV daily or on alternate days; 5–6 procedures up to 2 weeks until clinical response | Clinical response | |
| Specific types of poisoning | Removal of toxic substances with high protein-binding capacity and low distribution volume | Albumin, plasma | Gastric lavage, activated charcoal (depending on toxic substance); multiorgan support | 1–2 TPV daily until clinical response | Clinical response | |
| Systemic lupus erythematosus with severe vasculitic complications including lupus cerebritis and pneumonitis | Removal of autoantibodies | Albumin, plasma | Immunosuppression | 1–1.5 TPV daily or every other day, 3–6 sessions until clinical response | Clinical response | TPE is not indicated for the treatment of lupus nephritis |
TPE therapeutic plasma exchange, ICU intensive care unit, ANCA antineutrophil cytoplasmic antibody, HIT heparin-induced thrombocytopenia, IVIG intravenous immunoglobulins, GBM glomerular basement membrane, TPV total plasma volume, TTP thrombotic thrombocytopenic purpura, TMA thrombotic microangiopathy
aNot widely used yet and limited to a few specialized centers but strong evidence base in acute liver failure (especially hyperacute) in improving transplant free survival in patients who meet transplant criteria but are either ineligible for transplant or do not have access to timely transplant. TPE may also be used as a bridge to transplant in acute liver failure with multiple organ failure [75]