Organ or site
Diagnostic
(adequate for the diagnosis of cGvHD)
Distinctive
(seen in cGvHD but not aGvHD; insufficient to establish cGvHD diagnosis)
Other features
(cannot be used to establish a diagnosis)
Common
(seen with aGvHD and cGvHD)
Skin
Poikilodermia
Lichen-type features
Sclerotic features
Morphea-like features
Lichen sclerosis
Vitiligo
Sweat impairment
Ichtyosis
Keratosis pilaris
Decreased pigmentation
Increased pigmentation
Erythema
maculopapular rash
Puritus
Nails
Dystrophy
Longitudinal ridging, splitting, brittleness
Onycholysis
Pterygium
Destruction (usually symmetric, affects most nails)
Scalp and body hair
New alopecia (after recovery from chemoradiotherapy), scarring and nonscarring alopecia; scaling, papulosquamous lesions
Loss of body hair, typically patchy (including eyelashes, eyebrows)
Thinning scalp hair, coarse or dull (not due to endocrine or other causes)
Premature gray hair
Mouth
Lichen-type features
Hyperkeratotic plaques
Sclerosis with decreased range of motion
Xerostomia
Mucocele
Mucosal atrophy
Ulcers
Pseudomembranes
Gingivitis
Mucositis
Erythema
Pain
Eyes
New onset dry, gritty, or painful eyes
Cicatricial conjunctivitis
Keratoconjunctivitis sicca
Corneal ulceration
Excessive aqueous tearing
Photophobia
Periorbital hyperpigmentation
Blepharitis
Genitalia
Lichen-type features
Vaginal strictures or stenosis
Ulcers
Fissures
Erosion
GI tract
Esophageal webbing
Strictures or stenosis in the upper third of the esophagus
Pancreatic insufficiency
Anorexia
Nausea
Vomiting
Diarrhea
Weight loss
Failure to thrive
Liver
Total bilirubin, alk phos
2x ULN
ALT or AST
2x ULN
Lung
Bronchiolitis obiterans diagnosed with lung biopsy
Bronchiolitis obliterans diagnosed with PFTs and radiology
BOOP
Muscles
Fascia
Joints
Fasciitis
Joint stiffness of contractures secondary to sclerosis
Myositis or polymyositis (proximal muscle weakness; myalgia is uncommon)
Edema
Muscle cramps
Arthralgia or arthritis
Hematopoietic
Thrombocytopenia
Eosinophilia
Lymphopenia
Hypo- or hypergammaglobulinemia
Auto-antibodies (also AIHA, ITP)
Other
Pericardial or pleural effusions
Ascites
Peripheral neuropathy
Nephrotic syndrome
Myasthenia gravis
Cardiac conduction abnormality or cardiomyopathy
c.
Exclusion of other diagnoses
19.6 Grading of cGVHD
1.
According to the 2005 NIH consensus guidelines, cGVHD is graded on a scale of mild, moderate, or severe (Table 19.2):
Table 19.2
NIH cGHVD organ-specific staging form. (Source: Filopovich et al., BBMT 2005)
Score 0 | Score 1 | Score 2 | Score 3 | |
|---|---|---|---|---|
Performance score __________ KPS ECOG LPS | □ Asymptomatic and fully active (ECOG 0; KPS or LPS 100 %) | □ Symptomatic, fully ambulatory, restricted only in physically strenuous activity (ECOG 1, KPS or LPS 80–90 %) | □ Symptomatic, ambulatory, capable of self-care, > 50 % of waking hours out of bed (ECOG 2, KPS or LPS 60–70 %) | □ Symptomatic, limited self-care, > 50% of waking hours in bed (ECOG 3-4, KPS or LPS < 60 %) |
SKIN | □ No symptoms with no or minimal distinct signs | □< 18 % BSA with disease signs but no sclerotic features | □ 19–50 % BSA or superficial sclerotic features “not hidebound” (able to pinch) | □> 50 % BSA OR deep sclerotic features “hidebound” (unable to pinch) or interference with ADL due to impaired mobility, ulceration or severe pruritis |
Clinical features | ||||
□ Maculopapular rash | ||||
□ Lichen-type features | ||||
□ Papulosquamous or | ||||
icthyosis | ||||
□ Hyperpigmentation | ||||
□ Hypopigmentation | ||||
□ Keratosis pilaris | ||||
□ Erythema | ||||
□ Erythroderma | ||||
□ Polikiloderma | ||||
□ Sclerotic features | ||||
□ Pruritis | ||||
□ Hair | ||||
□ Nails | ||||
% BSA involved _____ | ||||
MOUTH | □ No symptoms with no or minimal distinct signs | □ Mild symptoms with disease signs but not limiting oral intake significantly | □ Moderate symptoms with signs with partial limitation of oral intake | □ Severe symptoms with disease signs on examination with major limitation of oral intake |
EYES | □ No symptoms | □ Mild dry eye symptoms not affecting ADL (requiring eye drops ≤ 3 x per day) or symptomatic signs of sicca keratitis | □ Moderate dry eye symptoms partially a affecting ADL (requiring drops > 3 x per day or punctal plugs), WITHOUT vision impairment | □ Severe dry eye symptoms significantly affecting ADL (special eyewear to relieve pain) or unable to work due to ocular symptoms or loss of vision caused by pseudomembranes or corneal ulceration |
Mean tear test (mm): | ||||
□> 10 | ||||
□6–10 | ||||
□≤ 5 | ||||
□Not done | ||||
GI TRACT | □ No symptoms | □ Symptoms such as dysphagia, anorexia, nausea, vomiting, abdominal pain or diarrhea without significant weight loss (< 5 %) | □ Symptoms associated with mild to moderate weight loss (5–15 %) | □ Symptoms associated with significant weight loss > 15 %, requires nutritional supplement for most calorie needs or esophageal dilation |
LIVER | □ Normal LFTs | □ Bilirubin, AP, AST or ALT < 2 × ULN | □ Bilirubin > 3 mg/dl or bili, enzymes 2–5 × ULN | □ Bili or enzymes > 5 x ULN |
LUNGS | □ No symptoms | □ Mild symptoms (dyspnea with stair climbing) | □ Moderate symptoms (dyspnea with level walking) | □ Severe symptoms (dyspnea at rest; requiring 02) |
□ FEV1/FVC ratio < 0.75 OR FEV1 of 51–75 %, without distinct findings of bronchiolitis obliterans (BO) on HRCT | □ FEV1/FVC ratio < 0.75 or FEV1 of 51–75 % with mild distinct findings of BO on HRCT | □ FEV1/FVC ratio <0.75 or FEV1 of 35–50% with distinct findings of BO on HRCT | □ FEV1/FVC ratio of < 0.75 % or FEV1 ≤ 34 % with distinct findings of BO on HRCT | |
JOINTS AND FASCIA | □ No symptoms | □ Mild tightness of arms or legs, normal or mild decreased ROM and not affecting ADL | □ Tightness of arms or legs or joint contractures erythema thought due to fasciitis, moderate decrease ROM and mild to moderate limitation of ADL | □ Contractures with significant decrease of ROM and significant limitation of ADL (unable to tie shoes, button shirts, dress self, etc.) |
GENITAL TRACT
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