1. Thrombotic Thrombocytopenic Purpura(TTP) Post -AllogeneicTransplant A haematological emergency: a nursing challenge
Janet Baker
Haem-Onc Daycare/Apheresis
Royal Marsden NHS Trust /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 good engraftment (platelets 242)
Day post-transplant clinic
Rash,forgetful,fever,poor oral intake
Creatinine LDH 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 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=

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
Fresh Frozen Plasma (FFP)
Cryo-Poor plasma
Solvent Detergent FFP
Cost per unit
£36
£42
£51
Volume per unit
mls
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. References
Journal of Clinical Apheresis:Clinical Applications of Therapeutic Apheresis,an evidence based approach.Volume 25.Issue
guidelines.org.uk
Marr H,et al.Successful treatment of transplant-associated microangiopathy with rituximab.New Zealand Medical Journal :1292

13. Questions?