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🌱 來自: Chronic GVHD

treatment of chronic graft-versus-host disease

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  • cGVHD, manifestations:

    • sclerotic cutaneous effects,
    • dry oral mucosa,
    • ulcerations and sclerosis of the gastrointestinal tract,
    • and elevated serum bilirubin.
  • See Clinical manifestations and diagnosis of chronic graft-versus-host disease

  • Grading is according to our modification of the National Institutes of Health NIH GVHD scoring system (form 1), which surveys symptoms, affected organ systems, and severity and extent of involvement (graded 0 to 3). There are no validated biomarkers for cGVHD severity. We classify cGVHD as:

  • Mild - ≤2 affected organs, no pulmonary manifestations, Eastern Cooperative Oncology Group (ECOG) performance status (PS) ≤2 (see ‘Mild cGVHD’ above)

  • Moderate - Involvement of ≥3 organs with score ≤1, lung score 1, any non-lung organ score 2, or ECOG PS ≥3 (see ‘Moderate cGVHD’ above)

  • Severe - Lung score ≥2, any non-lung score 3, or <100,000 platelets/microL (see ‘Severe cGVHD’ above):

  • Treatment is based on disease severity, scored with the modified NIH scale (algorithm 1):

  • Mild cGVHD - For patients with mild cGVHD, we suggest localized or topical treatment rather than systemic therapy (Grade 2C), to avoid adverse effects (AEs)/complications of systemic prednisone and other systemic immunosuppressive agents. (See ‘Mild cGVHD’ above.)

Localized or topical therapy varies according to the affected organ, as described above. (See ‘Localized/topical treatments’ above.)

  • Moderate cGVHD - For initial treatment of moderate cGVHD, we recommend systemic prednisone, rather than an alternative immunosuppressive agent, either alone or together with prednisone (Grade 2C). (See ‘Moderate cGVHD’ above.)

Topical therapy can be added to systemic prednisone, if needed, to ameliorate localized symptoms.

  • Severe cGVHD - For initial treatment of severe cGVHD, we suggest prednisone plus ruxolitinib, rather than prednisone alone or prednisone plus another second agent (Grade 2C). (See ‘Severe cGVHD’ above.)

  • Response assessment is performed according to the NIH Consensus Criteria for Clinical Trials in cGVHD. (See ‘Response assessment’ above.)

  • Steroid-refractory (SR) cGVHD - For patients with SR-cGVHD, we recommend addition of ruxolitinib to prednisone, rather than addition of other systemic medications (eg, cyclosporine, tacrolimus, ibrutinib) or non-pharmacologic approaches (eg, extracorporeal photopheresis, psoralen ultraviolet irradiation therapy) to prednisone (Grade 2B).