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GvHD bijlagen

Versie:
1.0
Publicatiedatum:
1 jun 2013
Auteur(s):
Ellen Meijer, Sacha Zeerleder
ID:
MATCH-GVB-013

1. Symptomatologie en scoren van cGvHD

Tabel 1. Signs and symptoms of cGvHD

AP indicates alkaline phosphatase; ALT, alanine aminotransferase; AIHA, autoimmune hemolytic anemia, ITP, idiopathic thrombocytopenic purpura.

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.

 

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
Sclerotic features
Morphea-like features
Lichen sclerosus-like features

Depigmentation

Papulosquamous lesions

Sweat impairment
Ichthyosis
Keratosis pilaris

Hypopigmentation Hyperpigmentation

Erythema

Maculopapular rash

Pruritus

Nails  

Dystrophy

Longitudinal ridging, splitting, or

brittle features

Onycholysis

Pterygium unguis

Nail loss (usually symmetric; affects most nails)

   
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)
Premature gray hair

 
Mouth Lichen planus-like features

Xerostomia

Mucoceles
Mucosal atrophy

Ulcers

Pseudomembranes

 

Gingivitis

Mucositis

Erythema

Pain

Eyes  

New onset dry, gritty, or

painful eyes
Cicatricial conjunctivitis

Keratoconjunctivitis sicca

Confluent areas of punctate keratopathy

Photophobia
Periorbital hyperpigmentation

Blepharitis (erythema of the eyelids with edema)

 
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
   
GI tract

Esophageal web

Strictures or

stenosis in the upper to mid third of the esophagus

  Exocrine pancreatic insufficiency

Anorexia
Nausea
Vomiting Diarrhea
Weight loss

Failure to thrive (infants and children)

Liver      

Total bilirubin, AP,

ALT > 2 x ULN

Lung

Bronchiolitis obliterans diagnosed with lung biopsy

BOS4

Air trapping and bronchiectasis on chest CT

Cryptogenic organizing pneumonia

Restrictive lung disease5

 

 
Muscles, fascia, joints

Fasciitis
Joint stiffness or

contractures secondary to fascitis or

sclerosis

Myositis or

polymyositis6

Edema
Muscle cramps
Arthralgia or arthritis
 
Hematopoietic and immune    

Thrombocytopenia

Eosinophilia

Lymphopenia
Hypo- or hypergammaglobulinemia

Autoantibodies (AIHA and ITP)

 
Other    

Raynaud’s phenomenon

Pericardial or pleural effusions
Ascites
Peripheral neuropathy

Nephrotic syndrome

Myasthenia gravis

Cardiac conduction abnormality or

cardiomyopathy

 

Tabel 2. Organ scoring of cGvHD

  SCORE 0 SCORE 1 SCORE 2 SCORE 3

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%)

SKIN1

Score % BSA

 

GVHD features to be scored by BSA:

Check all that apply:


☐ Maculopapular rash / 

     erythema

☐ Lichen planus-like

     features

☐ Sclerotic features

☐ Papulosquamous

     lesions or ichthyosis

☐ Kerastosis pilaris-like

     GVHD
 

☐ No BSA involved ☐ 118% BSA ☐ 19 – 50% BSA

☐ > 50% BSA

 

 Skin features score:

☐ No sclerotic features  

☐ Superficial sclerotic features “not

     hidebound” (able to pinch)

☐ Deep sclerotic

     features

     “Hidebound” (unable to pinch)

     Impaired mobility

     Ulceration

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

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

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

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)

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

 

 Lung score:

☐ FEV1 ≥ 80% ☐ FEV1 60 – 79% ☐ FEV1 40 – 59%

☐ FEV1 ≤ 39%

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.)

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

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)
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___

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.

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

Fase Klachten Dieet Tekenen intolerantie
Darmrust Darmkrampen
Grote hoeveelheid waterige diarree
Verlaagd albumine
Zeer korte passagetijd
Verminderde peristaltiek
Misselijkheid en braken
Oraal: NPOPV: volgens energie- en eiwitbehoefte  
Introductie orale (vloeibare) voeding Minimale krampen
Diarree < 500ml/24 uur
Passagetijd min. 1,5 uur
Niet-frequent misselijkheid en braken

Oraal: starten met 60 ml iedere 2-3 uur (enkele dagen) iso-osmotisch, laag-residu, lactosebeperkt

PV: volgens energie- en eiwitbehoefte

Toename ontlasting volume of diarree
Meer misselijkheid
Meer krampen
Introductie vast voedsel Minimale/geen krampen
Gevormde/vaste ontlasting
Oraal: introductie vast voedsel, iedere 3-4 uur:lactosearm, weinig vezels, weinig vet (20-40 g), niet-zuur, geen irriterende* voedingsmiddelen 

PV: volgens energie- en eiwitbehoefte

Zie boven
Uitbreiding dieet Minimale/geen krampen
Gevormde/vaste ontlasting
Oraal:
minimaal lactose, weinig vezels, niet-zuur, geen irriterende voedingsmiddelen, bij vetmalabsorptie: laag vet 

PV: benodigde aanvulling

Zie boven
Hervatten normaal dieet Geen krampen
Normale ontlasting
Normale passagetijd
Normaal albumine

Oraal: uitbreiden naar normaal dieet; per dag 1 nieuw product introduceren: zure producten alleen in combinatie met de maaltijd toevoegen, lactose, vezels. Volgorde afhankelijk van eigen voorkeur en tolerantie

Indien geen vetdiarree: vetconsumptie voorzichtig uitbreiden 

PV: stop zodra orale intake toereikend is

Zie boven
NPO: niets per os, PV: parenterale voeding
*irriterende voedingsmiddelen: zie onderstaand lijstje
Bron: FHCRC/SCCA guidelines long-term follow-up after HSCT http://www.fhcrc.org/science/clinical/ltfu/physician/physician.pdf

Irriterende producten:

  • Rauwe groenten en fruit
  • Gekruide producten
  • Vette dingen, zoals worst, gefrituurde producten
  • Peulvruchten
  • Citrusfruit (-sap)
  • Gedroogde vruchten (rozijnen, tutti frutti, enz.)
  • Tomatensoep
  • Gebonden soepen, gekruide soepen, soep met uit/bonen/bacon/chili/erwten

Introductie orale voeding

  1. Vloeibaar
    Starten met 1 kopje:

    • Thee (niet te sterk)
    • Appelsap (aangelengd met water)
    • Cranberrysap (aangelengd met water)
    • Bouillon
  2. Vast
    • Wit brood, beschuit, rijstcrackers,
    • Rijst, pasta, 1 lepel
    • Rice crispies / gepofte rijst
    • Fruit: ½ banaan, perzik of peer uit blik, geen rauw, vers fruit of andere fruitsoorten uit blik!
    • Gekookte wortelen of sperziebonen
    • Tonijn (kleine hoeveelheid)
    • Gekookt ei
    • Magere ham
    • Gestoomde vis
    • Kip, kalkoen
    • Aardappelen zonder schil

Aandachtspunten bij GvHD van het maagdarmkanaal

  • Controleer micronutriënten status (Zn, vitamine A, E, B1, B6, B12, foliumzuur, 25 OH D3); suppleer zonodig vitamines en mineralen
  • Overweeg consult diëtist voor vaststellen energie- en voedingsstoffenbehoefte e/o aanvullende diagnostiek
    • rustmetabolisme meten via indirecte calorimetrie
    • ontlastingsonderzoek kan meer inzicht in absorptieproblematiek geven; bomcalorimetrie via UMCG: 3 dagen ontlasting sparen en orale intake bijhouden.
    • g vet en stikstof in 24 u ontlasting, via klinische chemie VUmc
    • evt. nuchter citrulline of citrulline belastingstest
    • evt. Omegaven studie (pilot studie met intraveneuze visolie suppletie bij chronische GVHD-TD)
  • Overweeg inschakelen van nurse practitioner met aandachtsgebied GVHD voor het scoren van overige GVHD manifestaties, begeleiding en continuïteit.
  • Afhankelijk van de GVH activiteit/klachtenpatroon: parenterale of enterale bijvoeding geven. Zodra de acute fase voorbij is en een soort chronische fase ontstaat blijven veel patiënten ondervoed en voor enige tijd afhankelijk van kunstmatige voeding.

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)
   

 

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.

 

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