Canterbury DHB

Context

Chronic GvHD (cGvHD)

Chronic GvHD has been more difficult to study than aGvHD and in an attempt to correct this the NIH has recently sponsored a chronic GvHD consensus project. Pavletic et al 2006 has summarised the work of the cGvHD working parties and this is a valuable source of cGvHD information. cGvHD may occur to some extent in up to 80% of patients in the 2 years following SCT. Risk factors for the development of cGvHD include: prior acute GvHD, mismatch or MUD SCT, and peripheral blood as the source of stem cells.

In This Section

Clinical Features

Pathogenesis

Diagnosis and Grading and Severity Assessment

Histology of cGvHD

Management

Prognosis of cGvHD

Clinical Features

Pathogenesis

Diagnosis and Grading and Severity Assessment

See Pavletic 2006. The following criteria are based on the NIH Working Group recommendations:

cGvHD clinical features have been characterised as either diagnostic if they unequivocally indicate cGvHD or distinctive if they are merely suggestive of cGvHD.

Organ

Diagnostic features

Distinctive features

Skin

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

Depigmentation

Nails

 

Dystrophy, Longitudinal ridging, splitting, or brittle nails. Onycholysis Pterygium unguis. Nail loss.

Scalp, hair

 

New onset of scarring or nonscarring scalp alopecia. Scaling, papulosquamous lesions

Mouth

Lichen-type features

Hyperkeratotic plaques

Restriction of mouth opening

Xerostomia, Mucocele, Mucosal atrophy, Pseudomembranes

Ulcers

Eyes

 

New onset, dry, gritty, or painful eyes, Cicatricial conjunctivitis Keratoconjunctivitis sicca, punctuate keratopathy

Genitalia

Lichen planus-like features Vaginal scarring or stenosis

Erosions, Fissures, Ulcers

GI tract

Oesophageal web, strictures or stenosis in the oesophagus

 

Lung

Broncholitis obliterans (BO) diagnosed with lung biopsy

BO diagnosed with lung function tests and radiology

Muscles, joints

Fasciitis, joint stiffness or contractures

Myositis or polymyostitis

The diagnosis of cGvHD should be made if there is one diagnostic manifestation of cGvHD or at least one distinctive manifestation with the diagnosis confirmed by biopsy, other lab tests, or radiology in the same or another organ.

The extent of cGvHD should be scored as follows:

Extent and Severity of cGvHD

  • Score 0-3 to each organ based on severity of organ involvement
  • Mild chronic GvHD involves only 1 or 2 organs or sites (except the lung), with no clinically significant functional impairment (maximum of score 1 in all affected organs or sites.
  • Moderate chronic GvHD involves at least 1 organ or site with clinically significant but no major disability (maximum score of 2 in any affected organ or site) or 3 or more organs or sites with no clinically significant functional impairment (maximum score of 1 in all affected organs or sites). A lung score of 1 will also be considered moderate chronic GvHD.
  • Severe chronic GvHD indicates major disability caused by chronic GvHD (score of 3 in any organ or site). A lung score of 2 or greater will also be considered severe chronic GvHD.

Histology of cGvHD

See Shulmann et al for a recent review of the histological changes in cGvHD.

About this Canterbury DHB document (9077):

Document Owner:

Andrew Butler (see Who's Who)

Issue Date:

December 2016

Next Review:

December 2018

Keywords:

Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer

Topic Code: 9077