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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
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Objectives
 What is TTP?
 Post-Transplant TTP
 Case Study
 Nursing Challenges
 Discussion points
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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
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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
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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
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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
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Case Study
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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)
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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)
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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
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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
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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
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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
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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
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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?