Extracorporeal photopheresis for treatment of adults and children with acute GVHD: UK consensus statement and review of published literature

Abstract Background: Graft-versus-host disease (GVHD) is a serious complication with a significant negative impact on survival following allogeneic hematopoietic stem cell transplantation (HSCT) in both adults and children. Immunosuppressants like steroids remain the mainstay of treatment which carries risks of increased infection frequency, a negative impact on the graft-versus-leukemia effect, and an increased probability of secondary malignancies. Extracorporeal photopheresis (ECP) treatment, seems to ameliorate GVHD by immunomodulation, rather than immunosuppression. However it is difficult to perform in pediatric population. This is a retrospective review of 17 pediatric patients who underwent photopheresis with the Therakos Cellex system for graft-versus-host disease (GVHD). Procedure: Extracorporeal photopheresis regimen was same for all acute and chronic GVHD patients: initially every week (2 sessions/week) for two months, then every two weeks for two months; and finally, every month for at least one year. Demographic data, degree of GVHD before photopheresis, adverse reactions during photopheresis procedure, modification in immunosuppressive treatment, and response to photopheresis were recorded. Results: Acute GVHD occurred in seven patients, overlap and chronic GVHD occurred in five patients in each group. The improvement observed in eleven of twelve patients who have acute GVHD (90%) and in seven of ten chronic GVHD patients (70%). Thirteen patients had skin involvement before ECP and eleven of them responded to treatment (84%). Gastrointestinal involvement occurred in ten patients and seven of them improved during ECP treatment (70%). All of the four patient´s liver involvements failed to respond. No serious adverse reactions occurred. Conclusions: In conclusion, our study demonstrates that ECP with the Therakos Cellex system is a safe treatment option for GVHD in children. Disclosures No relevant conflicts of interest to declare.

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Extracorporeal photopheresis in prevention and treatment of acute GVHD

Transfusion and Apheresis Science ◽

10.1016/j.transci.2015.02.001 ◽

2015 ◽

Vol 52 (2) ◽

pp. 151-156 ◽

Cited By ~ 14

Author(s):

Carrie L. Kitko ◽

John E. Levine

Keyword(s):

Acute Gvhd ◽

Extracorporeal Photopheresis ◽

Prevention And Treatment

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An international multidisciplinary consensus statement on fasting before procedural sedation in adults and children

Anaesthesia ◽

10.1111/anae.14892 ◽

2020 ◽

Vol 75 (3) ◽

pp. 374-385 ◽

Cited By ~ 9

Author(s):

S. M. Green ◽

P. L. Leroy ◽

M. G. Roback ◽

M. G. Irwin ◽

G. Andolfatto ◽

...

Keyword(s):

Consensus Statement ◽

Procedural Sedation ◽

Adults And Children

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Extracorporeal photopheresis for the prevention of acute GVHD in patients undergoing standard myeloablative conditioning and allogeneic hematopoietic stem cell transplantation

Bone Marrow Transplantation ◽

10.1038/bmt.2009.307 ◽

2009 ◽

Vol 45 (6) ◽

pp. 1068-1076 ◽

Cited By ~ 27

Author(s):

P J Shaughnessy ◽

B J Bolwell ◽

K van Besien ◽

M Mistrik ◽

A Grigg ◽

...

Keyword(s):

Stem Cell ◽

Hematopoietic Stem Cell Transplantation ◽

Stem Cell Transplantation ◽

Cell Transplantation ◽

Hematopoietic Stem Cell ◽

Acute Gvhd ◽

Extracorporeal Photopheresis ◽

Myeloablative Conditioning ◽

Hematopoietic Stem

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International consensus on initial screening and follow-up of asymptomatic SDHx mutation carriers

Nature Reviews Endocrinology ◽

10.1038/s41574-021-00492-3 ◽

2021 ◽

Author(s):

Laurence Amar ◽

Karel Pacak ◽

Olivier Steichen ◽

Scott A. Akker ◽

Simon J. B. Aylwin ◽

...

Keyword(s):

Delphi Method ◽

Consensus Statement ◽

Consensus Algorithm ◽

Initial Screening ◽

International Consensus ◽

International Panel ◽

Mutation Carriers ◽

Adults And Children ◽

Near Future

AbstractApproximately 20% of patients diagnosed with a phaeochromocytoma or paraganglioma carry a germline mutation in one of the succinate dehydrogenase (SDHx) genes (SDHA, SDHB, SDHC and SDHD), which encode the four subunits of the SDH enzyme. When a pathogenic SDHx mutation is identified in an affected patient, genetic counselling is proposed for first-degree relatives. Optimal initial evaluation and follow-up of people who are asymptomatic but might carry SDHx mutations have not yet been agreed. Thus, we established an international consensus algorithm of clinical, biochemical and imaging screening at diagnosis and during surveillance for both adults and children. An international panel of 29 experts from 12 countries was assembled, and the Delphi method was used to reach a consensus on 41 statements. This Consensus Statement covers a range of topics, including age of first genetic testing, appropriate biochemical and imaging tests for initial tumour screening and follow-up, screening for rare SDHx-related tumours and management of elderly people who have an SDHx mutation. This Consensus Statement focuses on the management of asymptomatic SDHx mutation carriers and provides clinicians with much-needed guidance. The standardization of practice will enable prospective studies in the near future.

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