Treating TTP/HUS with plasma exchange: a single centre’s 25‐year experience

• Published in: British journal of haematology Vol. 143; no. 1; pp. 100 - 106
• Main Authors: Forzley, Brian R., Sontrop, Jessica M., Macnab, Jennifer J., Chen, Salina, Clark, William F.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01-10-2008; Blackwell
• Subjects: Adult; Biological and medical sciences; Canada; epidemiology; Female; haemolytic uraemic syndrome; Hematologic and hematopoietic diseases; Hemolytic-Uremic Syndrome - mortality; Hemolytic-Uremic Syndrome - therapy; Humans; Male; Medical sciences; Middle Aged; Nephrology. Urinary tract diseases; Nephropathies. Renovascular diseases. Renal failure; plasma exchange; Plasma Exchange - methods; plasmapheresis; Platelet diseases and coagulopathies; Purpura, Thrombotic Thrombocytopenic - mortality; Purpura, Thrombotic Thrombocytopenic - therapy; Recurrence; Renal failure; Retrospective Studies; Risk Factors; Survival Analysis; thrombotic thrombocytopenic purpura; Treatment Outcome; Canada; Kidney disease; Thrombocytopenia; Skin disease; Nervous system diseases; haemolytic uraemic syndrome; Urinary system disease; Hematology; Hemolytic uremic syndrome; Acute; Vascular disorders of the skin; Cardiovascular disease; Hemopathy; Epidemiology; Thrombohemolytic microangiopathy; Plasmapheresis; Vascular disease; Plasma exchange; Thrombotic thrombocytopenic purpura; Platelet; Hemolytic anemia; Treatment; Renal failure; Capillary vessel disease; Coagulopathy
• ISSN: 0007-1048; 1365-2141
• DOI: 10.1111/j.1365-2141.2008.07317.x

Summary
Thrombotic thrombocytopenic purpura/Haemolytic uremic syndrome (TTP/HUS) is a thrombotic microangiopathy with a 6‐month mortality rate of 16–29%. The present study described the clinical features, treatment regime and 6‐month all‐cause mortality rate of TTP/HUS patients at the London Health Sciences Centre (LHSC), Canada. Data for this retrospective cohort study were obtained from inpatient and outpatient records for all patients referred for plasma exchange therapy at LHSC, Canada between 1981 and 2006. Patients (n = 110) were categorized as: idiopathic primary (38%) or relapsed (16%), and secondary responsive (30%) or non‐responsive (16%). Mortality data were available for all but three patients. The all‐cause 6‐month mortality rate was 19% overall and was 12% and 26% among idiopathic and secondary TTP/HUS patients, respectively. No mortality events occurred among the 17 idiopathic patients who relapsed. Relapsed patients had the least severe presenting characteristics, the fastest response time, and experienced significant improvement in the severity of clinical features between the first and final presentation. These findings suggest an excellent outcome for relapsed TTP/HUS patients. Patient education, surveillance, and aggressive plasma exchange therapy are hypothesized to improve the likelihood of survival: these hypotheses should be tested in a randomized controlled trial.