Transcript Case Studies - the CBMTG 0801

Chronic Graft versus Host
Disease Examples
May 28, 2012
Example # 1
Diane is a 36 year old
On her clinic note you find the following:
•Maculopapular rash on her face and upper chest
(15% BSA)
•Food sensitivity, lichen planus-like oral changes
on physical exam
•Dry eyes; using eye drops twice a day
Example # 1
Does Diane have any diagnostic features of
chronic graft versus host disease?
Work through possible onset of chronic
GVHD worksheet…
Assessing Skin according to NIH Guidelines
Diagnositic:
 Poikiloderma
 Lichen planus-like features
 Sclerotic features
 Morphea-like features
Distinctive:
 Depigmentation
Common:
 Erythema (erythroderma)
 Pruritis
 Maculopapular rash
Additional features and/or reported symptoms:
 Ichthyosis
 Keratosis pilaris
 Hyperpigmentation
 Hypopigmentation
 Papulsquamous lesions
 Dry skin
 Limited mobility
Assessing Skin according to NIH Guidelines
Diagnositic:
 Poikiloderma
 Lichen planus-like features
 Sclerotic features
 Morphea-like features
Distinctive:
 Depigmentation
Common:
 Erythema (erythroderma)
 Pruritis
x Maculopapular rash
Additional features and/or reported symptoms:
 Ichthyosis
 Keratosis pilaris
 Hyperpigmentation
 Hypopigmentation
 Papulsquamous lesions
 Dry skin
 Limited mobility
Assessing Mouth according to NIH Guidelines
Diagnostic:
 Lichen-type features
 Hyperkeratotic plaques
 Restriction of mouth opening from sclerosis
Distinctive:
 Xerostomia (dry mouth)
 Mucocele
 Mucosal atrophy
 Pseudomembranes
 Ulcers
Common:
 Gingivitis
 Mucositis
 Erythema
 Pain
Additional features and/or reported symptoms:
 Chapped lips
 Bleeding gums
 Sensitivity to spicy foods, toothpaste, etc
Assessing Mouth according to NIH Guidelines
Diagnostic:
x Lichen-type features
 Hyperkeratotic plaques
 Restriction of mouth opening from sclerosis
Distinctive:
 Xerostomia (dry mouth)
 Mucocele
 Mucosal atrophy
 Pseudomembranes
 Ulcers
Common:
 Gingivitis
 Mucositis
 Erythema
 Pain
Additional features and/or reported symptoms:
 Chapped lips
 Bleeding gums
x Sensitivity to spicy foods, toothpaste, etc
Assessing Eyes According to the NIH Guidelines
Diagnostic: (None)
Distinctive:
 New onset dry, gritty, or painful eyes
 Cicatricial conjunctivitis
 Keratoconjunctivitis sicca
 Confluent areas of punctuate keratopathy
Common: (none)
Additional features and/or reported symptoms:
 Photophobia
 Periorbital hyperpigmentation
 Blepharitis
 Itchy eyes
 Difficulty opening eyes in the morning
 Excessive tearing
 Diminished visual acuity and/or blurring
Assessing Eyes According to the NIH Guidelines
Diagnostic: (None)
Distinctive:
x New onset dry, gritty, or painful eyes
 Cicatricial conjunctivitis
 Keratoconjunctivitis sicca
 Confluent areas of punctuate keratopathy
Common: (none)
Additional features and/or reported symptoms:
 Photophobia
 Periorbital hyperpigmentation
 Blepharitis
 Itchy eyes
 Difficulty opening eyes in the morning
 Excessive tearing
 Diminished visual acuity and/or blurring
Example # 1
Does Diane have Graft versus Host disease
Yes but…
Only the lichenoid changes in her mouth are diagnostic of
graft versus host disease
Once you have determined that the patient has diagnostic
features of graft versus host disease then complete cGVHD
scoring worksheet
Scoring Skin cGVHD
 0 No Symptoms
 1 <18% BSA with disease signs but NO sclerotic
features
 2 19-50% BSA OR involvement with superficial sclerotic
features “not hidebound” (able to pinch)
 3 >50% BSA OR deep sclerotic features “hidebound”
(unable to pinch) OR impaired mobility, ulceration or
severe pruritus
Calculating BSA and Rule of 9’s
Head / Neck
9%
Left upper extremity
Right upper extremity
Anterior torso
18 %
Posterior torso
18 %
Left lower extremity
Right lower extremity
Genitalia
4.5 % front; 4.5 % back
4.5 % front; 4.5 % back
9 % front; 9 % back
9 % front; 9 % back
1%
Scoring Skin cGVHD
 0 No Symptoms
X 1 <18% BSA with disease signs but NO sclerotic
features
 2 19-50% BSA OR involvement with superficial sclerotic
features “not hidebound” (able to pinch)
 3 >50% BSA OR deep sclerotic features “hidebound”
(unable to pinch) OR impaired mobility, ulceration or
severe pruritus
Calculating BSA and Rule of 9’s
Head / Neck
9%
Left upper extremity
Right upper extremity
Anterior torso
18 %
Posterior torso
18 %
Left lower extremity
Right lower extremity
Genitalia
4.5 % front; 4.5 % back
4.5 % front; 4.5 % back
9 % front; 9 % back
9 % front; 9 % back
1%
Scoring the Mouth according to NIH Guidelines
0 No Symptoms
1 Mild symptoms with disease signs but not limiting
oral intake significantly
2 Moderate symptoms with disease signs with
partial limitation of oral intake
3 >Severe symptoms with disease signs on
examination with major limitations of oral intake
Scoring the Mouth according to NIH Guidelines
0 No Symptoms
X 1 Mild symptoms with disease signs but not limiting
oral intake significantly
2 Moderate symptoms with disease signs with
partial limitation of oral intake
3 >Severe symptoms with disease signs on
examination with major limitations of oral intake
Scoring the Eyes according to NIH Guidelines
0 No Symptoms
1 Mild dry eye symptoms not affecting ADL (requiring drops <3 x per
day) OR asymptomatic signs of keratoconjunctivits sicca
2 Moderate dry eye symptoms affecting ADL (requiring drops >3
x per day or punctual plugs), WITHOUT vision impairment
3 Severe dry eye symptoms; significantly affecting ADL (special
eye are to relive pain) OR unable to work because of ocular
symptoms OR loss of vision caused by keratoconjunctivitis sicca
Scoring the Eyes according to NIH Guidelines
0 No Symptoms
X 1 Mild dry eye symptoms not affecting ADL (requiring drops <3 x per
day) OR asymptomatic signs of keratoconjunctivits sicca
2 Moderate dry eye symptoms affecting ADL (requiring drops >3
x per day or punctual plugs), WITHOUT vision impairment
3 Severe dry eye symptoms; significantly affecting ADL (special
eye are to relive pain) OR unable to work because of ocular
symptoms OR loss of vision caused by keratoconjunctivitis sicca
Summary of Example # 1
Diane has the following GVHD
scoring:
Skin score – 1
Mouth score – 1
Eyes score -1
Global Scoring of Chronic GVHD
Chronic GVHD Global Score at time
of this Assessment for Example # 1
Mild
Moderate
Severe
Chronic GVHD Global Score at time
of this Assessment for Example # 1
Mild
X Moderate
Severe
Example # 2
Julie is a 40 year old female
Six month cGVHD assessment – July 27, 2011
•On June 10 2011 noted that she started 50mg of
prednisone after seeing a respirologist for an FEV1 of 45%
and FEV/VC predicted and severe SOB on any exertion
•Respirologist opinion: bronchiolitis obliterans with
interstitial lung disease post stem cell transplant secondary
to graft versus host disease
•She has no other features of cGVHD in any other organ
Example # 2
• Does Julie have any diagnostic or distinctive
features of chronic graft versus host disease?
Assessing Lungs According to NIH Guidelines
Diagnostic:
 Bronchiolitis obliterans diagnosed with lung biopsy
Distinctive:
 Bronchiolitis obliterans diagnosed with PFTs & radiology
The following criteria must be present regarding BO:
 FEV in 1 second/forced vial capacity ratio <0.7 and FEV in 1 second 75% of
predicted.
 Evidence of air trapping or small airway thickening or bronchiectasis on highresolution chest CT scan (with inspiratory and expiratory cuts) residual
volume >120%, or pathologic confirmation of constrictive bronchiolitis.
 Absence of infection in the respiratory tract, documented with investigations
directed by clinical symptoms, such as radiologic studies (radiographs or
chest CT scans) or microbiologic cultures (sinus aspiration, URTI screen,
sputum culture, or bronchalveolar lavage).
Common:
 Bronchiolitis obliterans organizing pneumonia (BOOP)
Additional features and/or reported symptoms:
 Difficulty breathing
 Wheezing
 SOB at rest and/or exertion
 Dry cough
Assessing Lungs According to NIH Guidelines
Diagnostic:
 Bronchiolitis obliterans diagnosed with lung biopsy
Distinctive:
 Bronchiolitis obliterans diagnosed with PFTs & radiology
The following criteria must be present regarding BO:
X FEV in 1 second/forced vial capacity ratio <0.7 and FEV in 1 second 75%
of predicted.
 Evidence of air trapping or small airway thickening or bronchiectasis on highresolution chest CT scan (with inspiratory and expiratory cuts) residual
volume >120%, or pathologic confirmation of constrictive bronchiolitis.
 Absence of infection in the respiratory tract, documented with investigations
directed by clinical symptoms, such as radiologic studies (radiographs or
chest CT scans) or microbiologic cultures (sinus aspiration, URTI screen,
sputum culture, or bronchalveolar lavage).
Common:
 Bronchiolitis obliterans organizing pneumonia (BOOP)
Additional features and/or reported symptoms:
 Difficulty breathing
 Wheezing
 SOB at rest and/or exertion
 Dry cough
Example # 2
Issues:
•CT with inspiratory and expiratory cuts not done
•Lung biopsy not done
•No other diagnostic features of chronic graft
versus host disease
Therefore diagnosis of chronic graft versus host
disease can not be made.
Example # 2 Continues
1 year assessment:
Lung: FEV1 – 83%; SOB on walking on flat ground
Eyes: dry and gritty; Needs drops about 3-5 times per day
she can’t read a book
Mouth: Xerostomia; Erythema through the oral mucosa;
she feels like mouth has glue in it and has taste
disturbance with most foods
Liver: AST -92 (> 2 x ULN); ALT 109 (< 2 x ULN)
Assessing Lungs According to NIH Guidelines
Diagnostic:
 Bronchiolitis obliterans diagnosed with lung biopsy
Distinctive:
 Bronchiolitis obliterans diagnosed with PFTs & radiology
The following criteria must be present regarding BO:
 FEV in 1 second/forced vial capacity ratio <0.7 and FEV in 1 second 75% of
predicted.
 Evidence of air trapping or small airway thickening or bronchiectasis on highresolution chest CT scan (with inspiratory and expiratory cuts) residual
volume >120%, or pathologic confirmation of constrictive bronchiolitis.
 Absence of infection in the respiratory tract, documented with investigations
directed by clinical symptoms, such as radiologic studies (radiographs or
chest CT scans) or microbiologic cultures (sinus aspiration, URTI screen,
sputum culture, or bronchalveolar lavage).
Common:
 Bronchiolitis obliterans organizing pneumonia (BOOP)
Additional features and/or reported symptoms:
 Difficulty breathing
 Wheezing
 SOB at rest and/or exertion
 Dry cough
Assessing Lungs According to NIH Guidelines
Diagnostic:
 Bronchiolitis obliterans diagnosed with lung biopsy
Distinctive:
 Bronchiolitis obliterans diagnosed with PFTs & radiology
The following criteria must be present regarding BO:
 FEV in 1 second/forced vial capacity ratio <0.7 and FEV in 1 second 75% of
predicted.
 Evidence of air trapping or small airway thickening or bronchiectasis on highresolution chest CT scan (with inspiratory and expiratory cuts) residual
volume >120%, or pathologic confirmation of constrictive bronchiolitis.
 Absence of infection in the respiratory tract, documented with investigations
directed by clinical symptoms, such as radiologic studies (radiographs or
chest CT scans) or microbiologic cultures (sinus aspiration, URTI screen,
sputum culture, or bronchalveolar lavage).
Common:
 Bronchiolitis obliterans organizing pneumonia (BOOP)
Additional features and/or reported symptoms:
 Difficulty breathing
 Wheezing
X SOB at rest and/or exertion
 Dry cough
Assessing Eyes According to the NIH Guidelines
Diagnostic: (None)
Distinctive:
 New onset dry, gritty, or painful eyes
 Cicatricial conjunctivitis
 Keratoconjunctivitis sicca
 Confluent areas of punctuate keratopathy
Common: (none)
Additional features and/or reported symptoms:
 Photophobia
 Periorbital hyperpigmentation
 Blepharitis
 Itchy eyes
 Difficulty opening eyes in the morning
 Excessive tearing
 Diminished visual acuity and/or blurring
Assessing Eyes According to the NIH Guidelines
Diagnostic: (None)
Distinctive:
X New onset dry, gritty, or painful eyes
 Cicatricial conjunctivitis
 Keratoconjunctivitis sicca
 Confluent areas of punctuate keratopathy
Common: (none)
Additional features and/or reported symptoms:
 Photophobia
 Periorbital hyperpigmentation
 Blepharitis
 Itchy eyes
 Difficulty opening eyes in the morning
 Excessive tearing
 Diminished visual acuity and/or blurring
Assessing Mouth according to NIH Guidelines
Diagnostic:
 Lichen-type features
 Hyperkeratotic plaques
 Restriction of mouth opening from sclerosis
Distinctive:
 Xerostomia (dry mouth)
 Mucocele
 Mucosal atrophy
 Pseudomembranes
 Ulcers
Common:
 Gingivitis
 Mucositis
 Erythema
 Pain
Additional features and/or reported symptoms:
 Chapped lips
 Bleeding gums
 Sensitivity to spicy foods, toothpaste, etc
Assessing Mouth according to NIH Guidelines
Diagnostic:
 Lichen-type features
 Hyperkeratotic plaques
 Restriction of mouth opening from sclerosis
Distinctive:
X Xerostomia (dry mouth)
 Mucocele
 Mucosal atrophy
 Pseudomembranes
 Ulcers
Common:
 Gingivitis
 Mucositis
X Erythema
 Pain
Additional features and/or reported symptoms:
 Chapped lips
 Bleeding gums
X Sensitivity to spicy foods, toothpaste, etc
Assessing the Liver according to NIH Guidelines
Diagnostic: (none)
Distinctive: (none)
Common:
 Total bilirubin, alk phos >2x ULN
plus ALT or AST>2x ULN
Assessing the Liver according to NIH Guidelines
Diagnostic: (none)
Distinctive: (none)
Common:
X Total bilirubin, alk phos >2x ULN
plus ALT or AST>2x ULN
Example # 2 continues
Julie has some distinctive features of cGVHD but no
diagnostic features
Go back and review with primary physician
Further review with physician
shows…
She does have lichen-type features in her mouth…
Therefore, a diagnosis of chronic GVHD can be made
Scoring the Lungs according to NIH Guidelines
 0 No symptoms and/or FEV1> 80% OR LFS 3-5
 1 Mild symptoms (SOB after climbing one flight of stairs)
 2 Moderate symptoms (shortness of breath after
walking on flat ground) and/or FEV1 40-59%
 3 Severe symptoms (shortness of breath at rest
requiring oxygen)
Scoring the Lungs according to NIH Guidelines
 0 No symptoms and/or FEV1> 80% OR LFS 3-5
 1 Mild symptoms (SOB after climbing one flight of stairs)
X 2 Moderate symptoms (shortness of breath after
walking on flat ground) and/or FEV1 40-59%
 3 Severe symptoms (shortness of breath at rest;
requiring oxygen)
Scoring the Eyes according to NIH Guidelines
 0 No Symptoms
 1 Mild dry eye symptoms not affecting ADL (requiring drops
< 3 x per day) OR asymptomatic signs of keratoconjunctivits sicca
 2 Moderate dry eye symptoms affecting ADL (requiring drops >3
x per day or punctual plugs), WITHOUT vision impairment
 3 Severe dry eye symptoms; significantly affecting ADL (special
eye are to relive pain) OR unable to work because of ocular
symptoms OR loss of vision caused by keratoconjunctivitis sicca
Scoring the Eyes according to NIH Guidelines
 0 No Symptoms
 1 Mild dry eye symptoms not affecting ADL (requiring drops
< 3 x per day) OR asymptomatic signs of keratoconjunctivits sicca
X 2 Moderate dry eye symptoms affecting ADL (requiring drops >3
x per day or punctual plugs), WITHOUT vision impairment
 3 Severe dry eye symptoms; significantly affecting ADL (special
eye are to relive pain) OR unable to work because of ocular
symptoms OR loss of vision caused by keratoconjunctivitis sicca
Scoring the Mouth according to NIH Guidelines
 0 No Symptoms
 1 Mild symptoms with disease signs but not limiting
oral intake significantly
 2 Moderate symptoms with disease signs with
partial limitation of oral intake
 3 Severe symptoms with disease signs on
examination with major limitations of oral intake
Scoring the Mouth according to NIH Guidelines
 0 No Symptoms
 1 Mild symptoms with disease signs but not limiting
oral intake significantly
X 2 Moderate symptoms with disease signs with
partial limitation of oral intake
 3 Severe symptoms with disease signs on
examination with major limitations of oral intake
Scoring the Liver according to NIH Guidelines
 0 Normal LFTs
 1 One or more of the following elevated > 2 X ULN:
Total Bilirubin, Alk Phos, AST, ALT
 2 Total Bilirubin > 3 mg/dl (i.e. > 51 µmol/L)
OR
One or more of the following elevated 2-5 x ULN:
Total Bilirubin, Alk Phos, AST, ALT
 3 One or more of the following elevated > 5 x ULN:
Total Bilirubin, Alk Phos, AST, ALT
Scoring the Liver according to NIH Guidelines
 0
Normal LFTs
X 1 One or more of the following elevated > 2 X ULN:
Total Bilirubin, Alk Phos, AST, ALT
 2 Total Bilirubin > 3 mg/dl (i.e. > 51 µmol/L)
OR
One or more of the following elevated 2-5 x ULN:
Total Bilirubin, Alk Phos, AST, ALT
 3 One or more of the following elevated > 5 x ULN:
Total Bilirubin, Alk Phos, AST, ALT
Summary of Example # 2
Julie has the following GVHD scoring:
Lung score – 2
Eye score – 2
Mouth score – 2
Liver Score - 1
Global Scoring of Chronic GVHD
Chronic GVHD Global Score at time of
this Assessment for Example # 2
 Mild
 Moderate
 Severe
Chronic GVHD Global Score at time of
this Assessment for Example # 2
 Mild
 Moderate
X Severe
Example # 3
Mark is a 49 year old, 110 days post stem cell transplant
On his clinic note you find:
He has lichen type changes in his mouth; no taste
disturbance
Just admitted to hospital in the last 2 days for diarrhea 1.5
litres a day with nausea and vomiting. He has lost 10kg
and weighed 85kg at his last visit.
Assessing Mouth according to NIH Guidelines
Diagnostic:
 Lichen-type features
 Hyperkeratotic plaques
 Restriction of mouth opening from sclerosis
Distinctive:
 Xerostomia (dry mouth)
 Mucocele
 Mucosal atrophy
 Pseudomembranes
 Ulcers
Common:
 Gingivitis
 Mucositis
 Erythema
 Pain
Additional features and/or reported symptoms:
 Chapped lips
 Bleeding gums
 Sensitivity to spicy foods, toothpaste, etc
Assessing Mouth according to NIH Guidelines
Diagnostic:
X Lichen-type features
 Hyperkeratotic plaques
 Restriction of mouth opening from sclerosis
Distinctive:
 Xerostomia (dry mouth)
 Mucocele
 Mucosal atrophy
 Pseudomembranes
 Ulcers
Common:
 Gingivitis
 Mucositis
 Erythema
 Pain
Additional features and/or reported symptoms:
 Chapped lips
 Bleeding gums
 Sensitivity to spicy foods, toothpaste, etc
Assessing the GI Acute and Chronic
Chronic
Diagnostic:
 Esophageal web
 Strictures or stenosis in the upper
to mid third of the esophagus
Distinctive: (none)
Common:
 Anorexia
 Nausea
 Vomiting
X Diarrhea
X Weight loss
Additional features and/or reported
symptoms:
 Exocrine pancreatic insufficiency
Scoring the Mouth according to NIH Guidelines
 0 No Symptoms
 1 Mild symptoms with disease signs but not limiting oral intake
significantly
 2 Moderate symptoms with disease signs with partial limitation of
oral intake
 3 Severe symptoms with disease signs on examination with major
limitations of oral intake
Scoring the Mouth according to NIH Guidelines
 0 No Symptoms
X 1 Mild symptoms with disease signs but not limiting oral intake
significantly
 2 Moderate symptoms with disease signs with partial limitation of
oral intake
 3 Severe symptoms with disease signs on examination with major
limitations of oral intake
Scoring the GI according to NIH Guidelines
 0 No symptoms
 1 Symptoms such as dysphagia, anorexia, nausea,
vomiting, abdominal pain or diarrhea without
significant weight loss (< 5%)
 2 Symptoms associated with mild to moderate weight
loss (5-15%)
 3 Symptoms associated with significant weight loss >
15%, requires nutritional supplement for most calorie
needs OR esophageal dilation
Scoring the GI according to NIH Guidelines
 0 No symptoms
 1 Symptoms such as dysphagia, anorexia, nausea,
vomiting, abdominal pain or diarrhea without
significant weight loss (< 5%)
X
2 Symptoms associated with mild to moderate weight
loss (5-15%)
 3 Symptoms associated with significant weight loss >
15%, requires nutritional supplement for most calorie
needs OR esophageal dilation
Example # 3
Organ scores for chronic GVHD for Mark:
Mouth - 1
GI - 2
Global Scoring of Chronic GVHD
Chronic GVHD Global Score at time
of this Assessment for Example # 3
 Mild
 Moderate
 Severe
Chronic GVHD Global Score at time
of this Assessment for Example # 3
 Mild
X Moderate
 Severe
Example # 4
Doug is a 44 year old with the following findings at
his one year assessment
Sclerosis involving his arm (18% of skin involved)
and not able to extend his arm
Oral ulcers, unable to eat spicy foods
No other organs involved
Assessing Skin according to NIH Guidelines
Diagnositic:
 Poikiloderma
 Lichen planus-like features
 Sclerotic features
 Morphea-like features
Distinctive:
 Depigmentation
Common:
 Erythema (erythroderma)
 Pruritis
 Maculopapular rash
Additional features and/or reported symptoms:
 Ichthyosis
 Keratosis pilaris
 Hyperpigmentation
 Hypopigmentation
 Papulsquamous lesions
 Dry skin
 Limited mobility
Assessing Skin according to NIH Guidelines
Diagnositic:
 Poikiloderma
 Lichen planus-like features
X Sclerotic features
 Morphea-like features
Distinctive:
 Depigmentation
Common:
 Erythema (erythroderma)
 Pruritis
 Maculopapular rash
Additional features and/or reported symptoms:
 Ichthyosis
 Keratosis pilaris
 Hyperpigmentation
 Hypopigmentation
 Papulsquamous lesions
 Dry skin
X Limited mobility
Assessing Mouth according to NIH Guidelines
Diagnostic:
 Lichen-type features
 Hyperkeratotic plaques
 Restriction of mouth opening from sclerosis
Distinctive:
 Xerostomia (dry mouth)
 Mucocele
 Mucosal atrophy
 Pseudomembranes
 Ulcers
Common:
 Gingivitis
 Mucositis
 Erythema
 Pain
Additional features and/or reported symptoms:
 Chapped lips
 Bleeding gums
 Sensitivity to spicy foods, toothpaste, etc
Assessing Mouth according to NIH Guidelines
Diagnostic:
 Lichen-type features
 Hyperkeratotic plaques
 Restriction of mouth opening from sclerosis
Distinctive:
 Xerostomia (dry mouth)
 Mucocele
 Mucosal atrophy
 Pseudomembranes
X Ulcers
Common:
 Gingivitis
 Mucositis
 Erythema
 Pain
Additional features and/or reported symptoms:
 Chapped lips
 Bleeding gums
X Sensitivity to spicy foods, toothpaste, etc
Scoring Skin cGVHD
 0 No Symptoms
 1 <18% BSA with disease signs but NO sclerotic features
 2 19-50%BSA OR involvement with superficial sclerotic
features “not hidebound” (able to pinch)
 3 >50%BSA OR deep sclerotic features “hidebound” (unable
to pinch) OR impaired mobility, ulceration or severe pruritus
Calculating BSA and Rule of 9’s
Head / Neck 9 %
Left upper extremity
4.5 % front; 4.5 % back
Right upper extremity 4.5 % front; 4.5 % back
Anterior torso 18 %
Posterior torso
18 %
Left lower extremity
9 % front; 9 % back
Right lower extremity 9 % front; 9 % back
Genitalia
1%
Scoring Skin cGVHD
 0 No Symptoms
 1 <18% BSA with disease signs but NO sclerotic features
 2 19-50%BSA OR involvement with superficial sclerotic
features “not hidebound” (able to pinch)
X 3 >50%BSA OR deep sclerotic features “hidebound” (unable
to pinch) OR impaired mobility, ulceration or severe pruritus
Calculating BSA and Rule of 9’s
Head / Neck
9%
Left upper extremity
4.5 % front; 4.5 % back
Right upper extremity
4.5 % front; 4.5 % back
Anterior torso 18 %
Posterior torso 18 %
Left lower extremity
9 % front; 9 % back
Right lower extremity
9 % front; 9 % back
Genitalia
1%
Scoring the Mouth according to NIH Guidelines
0 No Symptoms
1 Mild symptoms with disease signs but not limiting oral intake
significantly
2 Moderate symptoms with disease signs with partial limitation of
oral intake
3 Severe symptoms with disease signs on examination with major
limitations of oral intake
Scoring the Mouth according to NIH Guidelines
0 No Symptoms
1 Mild symptoms with disease signs but not limiting oral intake
significantly
X 2 Moderate symptoms with disease signs with partial limitation of
oral intake
3 Severe symptoms with disease signs on examination with major
limitations of oral intake
Global Scoring of Chronic GVHD
Chronic GVHD Global Score at time of
this Assessment for Example # 4
 Mild
 Moderate
 Severe
Chronic GVHD Global Score at time of
this Assessment for Example # 4
 Mild
 Moderate
X Severe
Case Study # 5
• George is a 52 year old man who is 12 months post
HPCT
• He arrives for his Month 12 study visit
• Up until his arrival at the clinic there has been no
note of a diagnosis of chronic GVHD
• At the clinic visit the physical exam shows he has
significant contractures to his arms and the skin over
his shins is immobile (hidebound)
• George states his arms and shins have been that
way for 3 months
Does George have chronic GVHD?
Yes. (contractures and sclerosis/hidebound skin are diagnostic)
What is the date of onset (diagnosis)?
Month 9. (The patient is a reliable historian.)
Should the Part A & B Worksheet be completed
retrospectively for Month 9?
Yes. The GVHD Scoring Worksheet should also be completed.
Should the Part A & B Worksheet be completed at Month 12?
It is optional. The Study Visit Worksheet can be completed
instead. The GVHD Scoring Worksheet should be completed
(It is mandatory).
When should the Immunosuppressive Therapy Worksheet
be completed re: George’s visits?
Month 9 and Month 12.
Questions?