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The Royal Marsden Thrombotic Thrombocytopenic Purpura(TTP) Post -AllogeneicTransplant A haematological emergency: a nursing challenge Janet Baker Haem-Onc Daycare/Apheresis Royal Marsden NHS Trust 18/11/2011 1 2 The Royal Marsden Objectives What is TTP? Post-Transplant TTP Case Study Nursing Challenges Discussion points 3 The Royal Marsden What is TTP? Systemic thrombotic disease Mostly affecting small blood vessels Characterised by: - thrombocytopenia - elevated LDH - decreased haemoglobin - > 4 % red cell fragments on blood film May be neurological/ renal symptoms and fever 4 The Royal Marsden What causes TTP? Endothelial damage Platelet aggregation Red cell shearing and fragmentation Ischaemia to microvasculature,brain/lungs/kidneys-causing symptoms and potential organ failure TTP - spectrum of diseases? Congenital or acquired(post-pregnancy/viral) Auto-antibodies against ADAMTS 13 5 The Royal Marsden ADAMTS 13 A protein that regulates von Willebrand Factor(vWF) vWF circulates in plasma as large units(multimers) Helps platelets to adhere to vascular surfaces ADAMTS 13 breaks down large units Without it - excessive platelet aggregation Classic TTP -caused by deficiency of ADAMTS 13 Deficiency caused by auto-antibody (IgG) Blood test can detect antibody 6 The Royal Marsden Post-Transplant TTP ADAMTS 13 - no role Post-transplant MAHA (microangiopathic haemolytic anaemia) TAM (transplant associated microangiopathy) Endothelial damage caused by conditioning regimen(chemo/TBI)and/or Cyclosporin ? Other factors/GVHD Rare < 5% of all transplants …….but huge challenge Role of Therapeutic Plasma Exchange 7 The Royal Marsden Case Study Mary-21 year old female Natural Killer cell leukaemia Full Intensity MUD Cyclophosphamide/TBI/Alemtuzumab with Cyclosporine (CSA) Discharged Day + 18 - good engraftment (platelets 242) Day + 27 - post-transplant clinic Rash,forgetful,fever,poor oral intake Creatinine 240 LDH 750 Platelets 32 Admitted:CSA stopped / MMF started Seizures,renal and respiratory impairment Red cell fragments on blood film Transferred to Critical Care Unit Working diagnosis: TTP Day + 30 - daily Therapeutic Plasma Exchange (TPE) 8 The Royal Marsden Mary’s Story cont. Day 7 of TPE:Plts 13 LDH 700 : Refractory disease Other medications used: - steroids - Defibrotide - Vincristine x 2 - Cyclophosphamide x 1 - Rituximab x 4 Transferred from CCU to Transplant Unit Day + 52 Died Day + 78 : renal failure secondary to TTP 9 The Royal Marsden Mary’s Story cont. Mary had a total of 37 TPE procedures Blood counts day of death: - Platelets 11 (lowest 10) - Hgb 7.2 (lowest 6.9) - Wbc 0.3 - LDH 428 (highest 1395) Normal=98- 192 10 The Royal Marsden Points for Discussion Apheresis nurses:Role of TPE as first line therapy ? Evidence-base ASFA/BSCH guidelines Decision-making re:termination of TPE Patient/Family Advocate TPE: which Replacement Solution? Nursing Challenges 11 The Royal Marsden Plasma Replacement in TTP Fresh Frozen Plasma (FFP) Cryo-Poor plasma Solvent Detergent FFP Cost per unit £36 £42 £51 Volume per unit 250-300mls 250-300mls 200mls Content Contains near physiological levels of all plasma proteins Removal of cryoprecipitate which contains larger amounts of vWF Lower prion risk of vCJD transmission Less pathogens Lower patient reaction risk 12 The Royal Marsden References Journal of Clinical Apheresis:Clinical Applications of Therapeutic Apheresis,an evidence based approach.Volume 25.Issue 3 2010 http://www.transfusion guidelines.org.uk www.bcshguidelines.com Marr H,et al.Successful treatment of transplantassociated microangiopathy with rituximab.New Zealand Medical Journal.2009.122:1292 13 The Royal Marsden Questions? Does your centre perform TPE as first line therapy for Post transplant TTP? Does your centre give Rituximab as first line therapy. Do you use cryo poor plasma as replacement therapy if you use TPE? Do you use Octaplas as replacement therapy if you use TPE? Do Apheresis nurses play a part in the decision making process when considering starting and terminating TPE for post transplant TTP?