Chronic Graft Versus Host Disease

Reviewed and revised 29 August 2014

OVERVIEW

  • Chronic Graft vs Host Disease is organ dysfunction occurring > 100 days post transplantation
  • distinct clinical syndrome from acute GVHD
  • pathophysiology poorly understood
  • there can be an overlap period, where patients have both acute and chronic GVHD
  • affects 50% of HSCT recipients

CLINICAL FEATURES

  • chronic GVHD resembles spontaneously occurring autoimmune disorders, e.g. scleroderma
  • Organs typically  affected: skin (lichenoid and sclerotic rashes), mouth, joints, liver, eyes, gastrointestinal tract, and occasionally lungs

RISK FACTORS

  • older patient
  • CMV seropositive
  • male who receives a stem cell transplant from a multiparous woman

INVESTIGATIONS

  • tissue biopsies of skin, liver and stomach: apoptosis, infiltration of lymphocytes
  • investigations to exclude other conditions in the differential diagnosis

MANAGEMENT

  • Therapy relies on many of the same medications used to treat acute GVHD
  • patients require prolonged immunosuppressive treatment for an average of 2 to 3 years from the initial diagnosis
  • Glucocorticoids +/- calcineurin inhibitors (i.e., cyclosporine or tacrolimus) remain the standard initial treatment of chronic GVHD, but the outcomes are often not satisfactory, particularly for patients with high-risk features
  • Secondary options unproven: extracorporeal photopheresis, rituximab, sirolimus, mycofenolate mofetil, imatinib, pentostatin and infusion of mesenchymal stem cells

PROGNOSIS

  • can worsen survival due to more transplant-related mortality
  • infection from immunosuppression and disease-associated immune dysfunction accounts for most deaths
  • prolonged treatment is often required (e.g. months-to-years)
  • can also have a graft versus tumour effect

References and Links

LITFL

Journal articles

  • Carpenter PA. How I conduct a comprehensive chronic graft-versus-host disease assessment. Blood. 2011 Sep 8;118(10):2679-87. PubMed PMID: 21719600.
  • Inamoto Y, Flowers ME. Treatment of chronic graft-versus-host disease in 2011. Curr Opin Hematol. 2011 Nov;18(6):414-20. PMC3276600.

CCC 700 6

Critical Care

Compendium

Chris is an Intensivist and ECMO specialist at the Alfred ICU in Melbourne. He is also the Innovation Lead for the Australian Centre for Health Innovation at Alfred Health, a Clinical Adjunct Associate Professor at Monash University, and the Chair of the Australian and New Zealand Intensive Care Society (ANZICS) Education Committee. He is a co-founder of the Australia and New Zealand Clinician Educator Network (ANZCEN) and is the Lead for the ANZCEN Clinician Educator Incubator programme. He is on the Board of Directors for the Intensive Care Foundation and is a First Part Examiner for the College of Intensive Care Medicine. He is an internationally recognised Clinician Educator with a passion for helping clinicians learn and for improving the clinical performance of individuals and collectives.

After finishing his medical degree at the University of Auckland, he continued post-graduate training in New Zealand as well as Australia’s Northern Territory, Perth and Melbourne. He has completed fellowship training in both intensive care medicine and emergency medicine, as well as post-graduate training in biochemistry, clinical toxicology, clinical epidemiology, and health professional education.

He is actively involved in in using translational simulation to improve patient care and the design of processes and systems at Alfred Health. He coordinates the Alfred ICU’s education and simulation programmes and runs the unit’s education website, INTENSIVE.  He created the ‘Critically Ill Airway’ course and teaches on numerous courses around the world. He is one of the founders of the FOAM movement (Free Open-Access Medical education) and is co-creator of litfl.com, the RAGE podcast, the Resuscitology course, and the SMACC conference.

His one great achievement is being the father of two amazing children.

On Twitter, he is @precordialthump.

| INTENSIVE | RAGE | Resuscitology | SMACC

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