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Janet Baker Haem-Onc Daycare/Apheresis

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. Objectives. What is TTP?

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Janet Baker Haem-Onc Daycare/Apheresis

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  1. Thrombotic Thrombocytopenic Purpura(TTP) Post -AllogeneicTransplantA haematological emergency: a nursing challenge Janet Baker Haem-Onc Daycare/Apheresis Royal Marsden NHS Trust 18/11/2011

  2. Objectives What is TTP? Post-Transplant TTP Case Study Nursing Challenges Discussion points

  3. 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. 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. 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. 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. 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. 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. 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. 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. Plasma Replacement in TTP

  12. 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 transplant-associated microangiopathy with rituximab.New Zealand Medical Journal.2009.122:1292

  13. Questions?

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