TPE should only be carried out in conditions where there is good evidence of its effectiveness. The American Society for Apheresis [ASFA – //www.apheresis.org] produces regularly updated evidence-based guidelines [last updated in 2010]. Table 11.1 shows the 2010 category I ASFA indications for TPE [recommended as first-line therapy]. Category II indications [TPE is an established second-line therapy] are shown in Table 11.2. The evidence base is constantly developing and the decision to implement a course of TPE will usually involve discussion with a transfusion medicine specialist or other expert from the team providing the therapy.
Table 11.1 ASFA Category I indications for therapeutic plasma exchange [first-line therapy based on strong research evidence]
Speciality
Condition
Neurology
Acute Guillain–Barré syndrome
Chronic inflammatory demyelinating polyneuropathy
Myasthenia gravis
Polyneuropathy associated with paraproteinaemias
PANDASa
Haematology
Thrombotic thrombocytopenic purpura
Atypical haemolytic uraemic syndrome [autoantibody to factor H]
Hyperviscosity syndromes [paraproteinaemias]
Severe/symptomatic cryoglobulinaemia
Renal
Goodpasture’s syndrome [anti-glomerular basement membrane antibodies]
Antineutrophil cytoplasmic antibody [ANCA]-associated rapidly progressive glomerulonephritis
Recurrent focal segmental glomerular sclerosis
Antibody-mediated renal transplant rejection
Metabolic
Familial hypercholesterolaemia [homozygous]
Fulminant Wilson’s disease
a Paediatric autoimmune neuropsychiatric disorders associated with streptococcal infection.
Table 11.2 ASFA Category II indications for therapeutic plasma exchange [established second-line therapy]
Speciality
Condition
Neurology
Lambert–Eaton myasthenic syndrome
Acute exacerbation of multiple sclerosis
Chronic focal encephalitis
Neuromyelitis optica
Haematology
ABO-incompatible haemopoietic stem cell transplantation
Pure red cell aplasia
Life-threatening cold agglutinin disease
Atypical haemolytic uraemic syndrome [complement factor gene mutations]
Myeloma with cast nephropathy
Red cell alloimmunisation in pregnancy
Immunological
Catastrophic antiphospholipid syndrome
Cerebral systemic lupus erythematosus [SLE]
Metabolic
Refsum’s disease
11.1.2: Risks associated with therapeutic plasma exchange
Plasma exchange with albumin or saline causes a transient fall in blood-clotting factors and mild prolongation of prothrombin and activated partial thromboplastin times recovering in 4 to 24 hours. Clinically significant bleeding is rare but a coagulation screen should be undertaken before surgery or organ biopsy is performed. Other risks include haematomas at venepuncture/line insertion sites, vasovagal episodes with fainting, fluid overload or under-replacement, and allergic or anaphylactic reactions due to plasma infusion.
11.1.3: Thrombotic thrombocytopenic purpura [TTP]
This rare condition is a medical emergency with a mortality of 90% if untreated. It is caused by an acquired [autoimmune] or congenital deficiency of von Willebrand factor cleaving protein [ADAMTS13]. TPE, using FFP to replace ADAMTS13, is the treatment of choice and should be started as soon as possible after the diagnosis is suspected, ideally within 4–8 hours. The 2012 British Committee for Standards in Haematology [BCSH] Guidelines on the Diagnosis and Management of Thrombocytopenic Purpura and other Thrombotic Microangiopathies
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