Plasma exchange with solvent/detergent-treated plasma of resistant thrombotic thrombocytopenic purpura.
A patient with unremitting thrombotic thrombocytopenic purpura (TTP), with circulating von Willebrand factor (VWF) multimers of unusually high molecular weight, and refractory to standard plasma exchange therapy, was treated with solvent detergent (S/D) plasma. The patient achieved a sustained clinical and haematological remission, with normal VWF multimeric profile. Spontaneous remission of this patient's condition could not be excluded but would appear unlikely. S/D plasma was efficacious and potentially safe for repeated large-volume plasma exchange with respect to viral safety and reduction of anaphylactoid reactions. We have assayed coagulation factors and physiological inhibitors of haemostasis in S/D plasma, which were comparable to normal plasma except in the distribution of VWF multimers. The use of S/D plasma, previously reported in paediatric chronic relapsing TTP, should be assessed in further cases of adult TTP in the context of a clinical trial.
Harrison
C N
CN
Department of Haematology, University College Hospital, London.
Lawrie
A S
AS
Iqbal
A
A
Hunter
A
A
Machin
S J
SJ
D000328:Adult; D002908:Chronic Disease; D005260:Female; D006801:Humans; D010949:Plasma; D010951:Plasma Exchange / Q000379:methods*; D011697:Purpura, Thrombotic Thrombocytopenic / Q000628:therapy*; D014841:von Willebrand Factor / Q000032:analysis
D014841:von Willebrand Factor