Chronic Graft versus Host Disease Examples May 28, 2012.

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


Diagnositic: Poikiloderma Lichen planus-like features Sclerotic features Morphea-like features


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


Diagnostic:x Lichen-type features Hyperkeratotic plaques Restriction of mouth opening from sclerosis



Additional features and/or reported symptoms: Chapped lips Bleeding gumsx 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


Diagnostic: (None)

Distinctive:x New onset dry, gritty, or painful eyes Cicatricial conjunctivitis Keratoconjunctivitis sicca Confluent areas of punctuate keratopathy

Common: (none)


Example # 1Does 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 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

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


Scoring the Mouth according to NIH Guidelines

0 No Symptoms1 Mild symptoms with disease signs but not limiting

oral intake significantly2 Moderate symptoms with disease signs with

partial limitation of oral intake3 >Severe symptoms with disease signs on

examination with major limitations of oral intake


0 No Symptoms

X 1 Mild symptoms with disease signs but not limiting

oral intake significantly2 Moderate symptoms with disease signs with

partial limitation of oral intake3 >Severe symptoms with disease signs on


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


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

MildModerateSevere


Mild

X ModerateSevere

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 high-resolution 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




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.





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)





predicted.









predicted.




Additional features and/or reported symptoms: Difficulty breathing WheezingX SOB at rest and/or exertion Dry cough


Diagnostic: (None)

Distinctive: New onset dry, gritty, or painful eyes Cicatricial conjunctivitis Keratoconjunctivitis sicca Confluent areas of punctuate keratopathy

Common: (none)



Diagnostic: (None)

Distinctive:X New onset dry, gritty, or painful eyes Cicatricial conjunctivitis Keratoconjunctivitis sicca Confluent areas of punctuate keratopathy

Common: (none)




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 gumsX 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)


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


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


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



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


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


Mild Moderate Severe


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 GuidelinesDiagnostic:X Lichen-type features Hyperkeratotic plaques Restriction of mouth opening from sclerosis




Assessing the GI Acute and Chronic

ChronicDiagnostic: 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


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


0 No Symptoms

X 1 Mild symptoms with disease signs but not limiting oral intake

significantly


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


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


Diagnositic:

Poikiloderma Lichen planus-like features Sclerotic features Morphea-like features



Additional features and/or reported symptoms:

Ichthyosis Keratosis pilaris Hyperpigmentation Hypopigmentation Papulsquamous lesions Dry skin Limited mobility


Diagnositic:

Poikiloderma Lichen planus-like featuresX Sclerotic features Morphea-like features



Additional features and/or reported symptoms:

Ichthyosis Keratosis pilaris Hyperpigmentation Hypopigmentation Papulsquamous lesions Dry skinX Limited mobility

Assessing Mouth according to NIH GuidelinesDiagnostic: Lichen-type features Hyperkeratotic plaques Restriction of mouth opening from sclerosis






Distinctive: Xerostomia (dry mouth) Mucocele Mucosal atrophy PseudomembranesX Ulcers


Additional features and/or reported symptoms: Chapped lips Bleeding gumsX Sensitivity to spicy foods, toothpaste, etc

Scoring Skin cGVHD

0 No Symptoms


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


Scoring Skin cGVHD

0 No Symptoms


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



0 No Symptoms


significantly


oral intake




0 No Symptoms


significantly

X 2 Moderate symptoms with disease signs with partial limitation of

oral intake




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)

Should the Part A & B Worksheet be completed retrospectively for Month 9? Yes. The GVHD Scoring Worksheet should also be completed.

What is the date of onset (diagnosis)? Month 9. (The patient is a reliable historian.)

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?

Chronic Graft versus Host Disease Examples May 28, 2012.

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