1. Symptomatologie en scoren van cGvHD
Tabel 1. Signs and symptoms of cGvHD
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AP indicates alkaline phosphatase; ALT, alanine aminotransferase; AIHA, autoimmune hemolytic anemia, ITP, idiopathic thrombocytopenic purpura. 1 In all cases, infection, drug effect, malignancy, or other causes must be excluded. 2 Can be acknowledged as part of the chronic GVHD manifestations if diagnosis is confirmed. 3 Common refers to shared features by both acute and chronic GVHD. 4 BOS can be diagnostic for lung chronic GVHD only if distinctive sign or symptom present in another organ (see text). 5 Pulmonary entities under investigation or unclassified. 6 Diagnosis of chronic GVHD requires biopsy.
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| Organ or Site | Diagnostic (Sufficient to Establish the Diagnosis of Chronic GvHD) | Distinctive1 (Seen in Chronic GVHD, but Insufficient Alone to Establish a Diagnosis of Chronic GvHD) |
Other Features or Unclassified Entities2 |
Common (Seen with Both Acute and Chronic GvHD)3 |
| Skin |
Poikiloderma Lichen planus-like features |
Depigmentation Papulosquamous lesions |
Sweat impairment Hypopigmentation Hyperpigmentation |
Erythema Maculopapular rash Pruritus |
| Nails |
Dystrophy Longitudinal ridging, splitting, or brittle features Onycholysis Pterygium unguis Nail loss (usually symmetric; affects most nails) |
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| Scalp and body hair |
New onset of scarring or nonscarring scalp alopecia (after recovery from chemoradiotherapy) Loss of body hair Scaling |
Thinning scalp hair, typically patchy, coarse, or dull (not explained by endocrine or other causes) |
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| Mouth | Lichen planus-like features |
Xerostomia Mucoceles Ulcers Pseudomembranes |
Gingivitis Mucositis Erythema Pain |
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| Eyes |
New onset dry, gritty, or painful eyes Keratoconjunctivitis sicca Confluent areas of punctate keratopathy |
Photophobia Blepharitis (erythema of the eyelids with edema) |
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| Genitalia |
Lichen planus-like features Lichen sclerosus-like features Females: Vaginal scarring or clitoral / labial agglutination Males: Phimosis or urethral / meatus scarring or stenosis |
Erosions Fissures Ulcers |
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| GI tract |
Esophageal web Strictures or stenosis in the upper to mid third of the esophagus |
Exocrine pancreatic insufficiency |
Anorexia Failure to thrive (infants and children) |
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| Liver |
Total bilirubin, AP, ALT > 2 x ULN |
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| Lung |
Bronchiolitis obliterans diagnosed with lung biopsy BOS4 |
Air trapping and bronchiectasis on chest CT |
Cryptogenic organizing pneumonia Restrictive lung disease5
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| Muscles, fascia, joints |
Fasciitis contractures secondary to fascitis or sclerosis |
Myositis or polymyositis6 |
Edema Muscle cramps Arthralgia or arthritis |
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| Hematopoietic and immune |
Thrombocytopenia Eosinophilia Lymphopenia Autoantibodies (AIHA and ITP) |
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| Other |
Raynaud’s phenomenon Pericardial or pleural effusions Nephrotic syndrome Myasthenia gravis Cardiac conduction abnormality or cardiomyopathy |
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Tabel 2. Organ scoring of cGvHD
| SCORE 0 | SCORE 1 | SCORE 2 | SCORE 3 | |||
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PERFORMANCESCORE:
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 selfcare, > 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%) |
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SKIN1 Score % BSA
GVHD features to be scored by BSA: Check all that apply:
erythema ☐ Lichen planus-like features ☐ Sclerotic features ☐ Papulosquamous lesions or ichthyosis ☐ Kerastosis pilaris-like GVHD |
☐ No BSA involved | ☐ 1 – 18% BSA | ☐ 19 – 50% BSA |
☐ > 50% BSA
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Skin features score: |
☐ No sclerotic features |
☐ Superficial sclerotic features “not hidebound” (able to pinch) |
☐ Deep sclerotic features “Hidebound” (unable to pinch) Impaired mobility Ulceration |
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| MOUTH | ☐ No symptoms |
☐ Mild symptoms with disease signs, but not limiting oral intake significantly |
☐ Moderate symptoms with disease signs with partial limitation of oral intake |
☐ Severe symptoms with disease signs on examination with major limitation of oral intake |
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| EYES |
☐ No symptoms |
☐ Mild dry eye symptoms, not affecting ADL (requirement of lubricant eyedrops ≤ 3 x per day) |
☐ Moderate dry eye symptoms, partially affecting ADL (requiring lubricanting eyedrops > 3 x per day or punctal plugs), WITHOUT new visionimpairment due to KCS |
☐ Severe dry eye symptoms, significantly affecting ADL (special eyeware to relieve pain) OR unable to work, because of ocular symptoms OR loss of vision due to KCS |
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| GI TRACT | ☐ No symptoms |
☐ Symptoms without significant weight loss (< 5%)2 |
☐ Symptoms associated with mild to moderate weight loss (5 – 15%) OR moderate diarrhea without significant interference with daily living |
☐ Symptoms associated with significant weight loss > 15%, requires nutritional supplement for most calorie needs OR esophageal dilation OR severe diarrhea with significant interference with daily living |
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| LIVER |
☐ Normal total bilirubin and ALT or AP < 3 x ULN |
☐ Normal total bilirubin with ALT ≥ 3 – 5 x ULN or AP ≥ 3 x ULN |
☐ Elevated total bilirubin, but ≤ 3 mg/dl (≤ 50 µmol/L) or ALT > 5 x ULN |
☐ Elevated total bilirubin > 3 mg/dl (> 50 µmol/L) |
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LUNGS3 Symptom score: |
☐ No symptoms |
☐ Mild symptoms (shortness of breath after climbing one flight of steps) |
☐ Moderate symptoms (shortness of breath after walking on flat ground) |
☐ Severe symptoms (shortness of breath at rest; requiring O2
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Lung score: |
☐ FEV1 ≥ 80% | ☐ FEV1 60 – 79% | ☐ FEV1 40 – 59% |
☐ FEV1 ≤ 39% |
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| JOINTS AND FASCIA | ☐ No symptoms |
☐ Mild tightness of arms or legs, normal or mild decreased range of motion (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.) |
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| GENITAL TRACT | ☐ No symptoms |
☐ Mild signs4and females with or without discomfort on gynecologic exam |
☐ Moderate signs and may have symptoms with discomfort on gynecologic exam |
☐ Severe signs with or without symptoms | ||
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OVERALL GVHD SEVERITY (Opinion of the evaluator) |
☐ No GVHD | ☐ Mild | ☐ Moderate | ☐ Severe | ||
| Other indicators, clinical manifestations or complications related to chronic GVHD (check all that apply and assign a score to its severity (0 – 3) based on its functional impact where applicable (none – 0, mild – 1, moderate – 2, severe – 3) |
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| Ascites (serositis)___ Myasthenia Gravis___ Polymyositis___ Platelets < 100,000/μl ___ |
Pericardial Effusion___ Nephrotic syndrome___ Weight loss > 5% without GI symptoms___ |
Pleural Effusion(s)___ Peripheral Neuropathy___ Eosinophilia > 500μl___ Other___ |
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ECOG indicates Eastern Cooperative Oncology Group; KPS, Karnofsky Performance Status; LPS, Lansky Performance Status; BSA, body surface area; ADL, activities of daily living; KCS, keratoconjunctivitis sicca; AP, alkaline phosphatase; ALT, alanine aminotransferase; ULN, normal upper limit. 1 Skin scoring should use both percentage of BSA involved by disease signs and the cutaneous features scales. When a discrepancy exists between the percentage of total body surface (BSA) score and the skin feature score, OR if superficial sclerotic features are present (Score 2), but there is impaired mobility or ulceration (Score 3), the higher level should be used for the final skin scoring. 2 Weight loss within 3 months. 3 Lung scoring should be performed using both the symptoms and FEV1 scores whenever possible. FEV1 should be used in the final lung scoring where there is discrepancy between symptoms and FEV1 scores. 4 To be completed by specialist or trained medical providers. |
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Tabel 3. Huidpercentages GvHD*
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Erythema-teuze rash ACTIEF |
Erythema-teuze rash NIET ACTIEF |
Moveable sclerose | Non-moveable sclerose / subcutane sclerose of fasciitis | |||||||||||
| Lichaamsdeel | % regio | X* | Totaal | % regio | X* | Totaal | % regio | X* | Totaal | % regio | X* | Totaal | ||
| Hoofd / nek / behaarde hoofdhuid | 0 | 0.09 | 0 | 0 | 0.09 | 0 | 0 | 0.09 | 0 | 0 | 0.09 | 0 | ||
| Voorzijde romp | 0 | 0.18 | 0 | 0 | 0.18 | 0 | 0 | 0.18 | 0 | 0 | 0.18 | 0 | ||
| Rug | 0 | 0.18 | 0 | 0 | 0.18 | 0 | 0 | 0.18 | 0 | 0 | 0.18 | 0 | ||
| Linker arm | 0 | 0.09 | 0 | 0 | 0.09 | 0 | 0 | 0.09 | 0 | 0 | 0.09 | 0 | ||
| Rechter arm | 0 | 0.09 | 0 | 0 | 0.09 | 0 | 0 | 0.09 | 0 | 0 | 0.09 | 0 | ||
| Linker been | 0 | 0.18 | 0 | 0 | 0.18 | 0 | 0 | 0.18 | 0 | 0 | 0.18 | 0 | ||
| Rechter been | 0 | 0.18 | 0 | 0 | 0.18 | 0 | 0 | 0.18 | 0 | 0 | 0.18 | 0 | ||
| Genitalia | 0 | 0.01 | 0 | 0 | 0.01 | 0 | 0 | 0.01 | 0 | 0 | 0.01 | 0 | ||
| Totaal | ||||||||||||||
* Interactieve berekening huidpercentages: Huidpercentages GvHD
2. Acute GvHD response definitions
- Complete response:
The return of acute GvHD to grade O - Partial response:
Improvement of at least 1 organ, with no worsening in other organs - Mixed response:
Improvement of at least 1 organ, with worsening in at least 1 other organ - Stable disease:
No significant change in any organ system - Progressive disease:
Progression in at least 1 organ system without improvement in any other organs.
3. Voedingsschema bij Graft-versus-Hostziekte van het maagdarmkanaal
Zie de folder: Dieetadviezen bij graft versus host ziekt van de de darm
4. Bereidingsvoorschriften
| R/ dexamethason mondspoeling 0,11 mg/ml (voorschrift voor 1000 ml). | ||
| 1. dexamethasoni natrii phosphas 0.00 H20 Ph.Eur. | 143,0 | mg |
| 2. natrii metabisulfis 0.00 H20 Ph.Ned.8 | 50,0 | mg |
| 3. natrii edetas 2.00 H20 Ph.Eur. | 500,0 | mg |
| 4. sorbitolum 70 per centum cristallisabile Ph.Eur. | 333,0 | mg |
| 5. essence frambozen huisnorm | 1,0 | ml |
| 6. solutio methylparabeni conc FNA huisnorm | 6,70 | ml |
| 7. aqua ad injectabilia Ph.Eur. | ad 1000 | ml |
| Da flacon voor mondspoeling | ||
| S/ 6 dd 10 cc, 5 minuten spoelen en uitspugen. (Maximale bewaartermijn: 6 maanden) |
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| R/ | Protopic 0.1% of 0.03% | 24 | g |
| Hypromellose 4000 mpa.s | 6 | g | |
| S/ | mondzalf, tube 30 g, No:….. | ||
| Gebruik: 3 dd appliceren, met vinger (wang, tandvlees, tongrand, lippen), nadien ½ uur niet drinken of eten. | |||
Hierbij machtigingsverzoek indienen voor ziektekostenverzekering.
Wijziging t.o.v. vorige versie
220324: voedingsschema bij GvH van het maagdarmkanaal. Tekst verwijdert. Folder toegevoegd. (M. Gillissen, M.Wondergem, I. Dekker)