Transcript Photopheresis in a Pediatric Population

Photopheresis in a
Pediatric Population
MARY ANN MICHAEL, MSN, RN, CNP
Objectives
 Review the differences of acute and chronic Graft vs.
Host (GVHD) Disease.
 Discuss standard treatment of GVHD disease.
 Illustrate the use of extracorporeal photopheresis
(ECP) and it’s implementation in a pediatric setting.
 Discuss pediatric experience using ECP for treatment
of GVHD at Cincinnati Children’s Hospital.
GVHD
Graft vs. Host Disease is caused by an immune
response that occurs when the donor’s T lymphocytes
(graft) recognizes the patient’s tissues (host) as foreign
and attacks.
GVHD
Chronic
Acute
 Skin
 Skin, nails
 Liver
 GI tract
 GI tract
 Liver
 Eyes
 Mouth
 Lungs
 Muscle, fascia, joints
Acute GVHD: Skin
Acute GVHD: Skin
Acute GVHD: Skin
Acute GVHD: Skin
Acute GVHD: Skin
Acute GVHD: Skin
Acute GVHD: Skin
Acute GVHD: Gut
STAGE
*use
ml/day for adult patients and ml/ms/day for pediatric patients
Skin
Liver (Bilirubin)
Intestinal tract*
(Diarrhea)
Stage 0
No rash
<2.0 mg/dL or
<3.5mmol/L
None or
<500 ml/day or
<280 ml/m2/day
Stage 1
Maculopapular
rash, <25% of body
surface
2.0-3.0 mg/dL or
35-052 mmol/L
>500 but <1000
ml/day or 2880-555
ml/m2/day
Stage 2
Maculopapular
rash, 25-50% of
body surface
3.1-6.0 mg/dL or
53-103 mmol/L
>1000 but <15000
ml/day or 556-833
ml/m2/day
Stage 3
Generalized
erythroderma
6.1-15.0 mg/dL or
104-256 mmol/L
>1500 ml/day or
>833 ml/m2/day
Stage 4
Generalized
erythroderma with
bullae formation
and desquamation
>15.0 md/dL or
>256mmol/L
Severe abdominal
pain, with or without
ileus
OVERALL GRADE
Skin
Liver
Intestinal tract
Grade 0
Stage 0
Stage 0
Stage 0
Grade I
Stage 1-2
None
None
Grade II
Stage 3 or
Stage 1 or
Stage 1
Stage 2-3 or
Stage 2-4
Stage 4
-
Grade III
Grade IV
Stage 4 or
Standard Treatment
Acute GVHD
Chronic GVHD
 Steroids
 Cyclosporine or
 Steroids
 Cyclosporine or
 Alemtuzumab (Campath)
 Alemtuzumab (Campath)
 Photopheresis
 Photopheresis
Tacrolimus
 Mycophenolate mofetil
or other monoclonal
antibody therapy
Tacrolimus
or other monoclonal
antibody therapy
Chronic graft versus host disease (cGVHD)
• Chronic GVHD and acute GVHD are different but
related diseases.
• However, the strongest predictor of getting cGVHD is
having had aGVHD.
• Refractory GVHD is defined as progressive disease
after a minimum of 7 days of 2 mg/kg/day steroid
treatment.
Chronic graft versus host disease (cGVHD)
 A complication that occurs in about 40% of
allogeneic transplant patients.
 Usually occurs > 100 days post transplant.
 Some patients may develop features of both acute
and chronic GVHD.
 Death from GVHD is usually related to infection.
•
Used when traditional
therapy has failed.
Used concurrently with
traditional therapy.
•
Whole blood is and
collected and separated
in a centrifuge. Buffy
coat is treated with 8methoxypsoralen
(Uvadex), exposed to
ultraviolet light, then is
reinfused.
•
Our current thought is
that during exposure to
UV light, the damaged
T-lymphocytes die off,
the immune system
recognizes the dying
abnormal cells and
begin to produce
healthy lymphocytes.
Photopheresis
Photopheresis Considerations
Disadvantages
 Requirement of frequent
treatments at the
hospital.
 Willingness to comply
with long term therapy
(months to years).
 Cost ( about $4000 or
€5200).
 Indwelling apheresis line
placement.
Advantages
 Ability to wean
steroids.
 Treatments are
generally well
tolerated.
 Preserves lymphocytes,
thus the ability to fight
infections.
One of Our Early Patients - JT
•
•
•
•
•
•
Male
DOB 8/8/2003
Diagnosis: NEMO – skin
GVHD
Allogeneic matched
unrelated transplant (8/8
match) 12/2006
Began photopheresis on
5/28/2008
Successfully weaned off
steroid therapy.
JT Pre-Treatment
JT After Years of Treatment
Our Experience
 Photopheresis was started at Cincinnati Children’s
Hospital for treatment of GVHD in 2008.
 18 patients receiving photopheresis therapy.
 All patients had allogeneic transplants with matched
unrelated donors.
 Number of treatments 10->200.
250
200
150
NUMBER OF
TREATMENTS
100
50
0
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
PATIENTS
TREATED
AT CCHMC
SKIN
LUNG
LIVER
COMBINATION
Conclusion
 Photopheresis is an approved treatment of GVHD.
 Literature supports the use of photopheresis in
treatment of GVHD and suggests early initiation of
therapy.
 Treatment is well tolerated in the pediatric
population.
 Deaths were not resultant of treatment.
A Special Thank You . . .
 Stella Davies, MD for all of her help and support.
 Mark Mueller, RN for his help with photography.
 Kelly Anstead, RN a Hoxworth Blood Center
pheresis nurse, for her wealth of knowledge.
 Carrie Gifford, Business Manager for Cancer and
Blood Disease Institute, for her help with data
retrieval.