prikaz prve stranice dokumenta Diagnosis and Management of Secondary HLH/MAS Following HSCT and CAR-T Cell Therapy in Adults; A Review of the Literature and a Survey of Practice Within EBMT Centres on Behalf of the Autoimmune Diseases Working Party (ADWP) and Transplant Complications W
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Scientific paper - Original scientific paper
Diagnosis and Management of Secondary HLH/MAS Following HSCT and CAR-T Cell Therapy in Adults; A Review of the Literature and a Survey of Practice Within EBMT Centres on Behalf of the Autoimmune Diseases Working Party (ADWP) and Transplant Complications Working Party (TCWP)
Frontiers in Immunology, 11 (2020); 524. https://doi.org/10.3389/fimmu.2020.00524
Sandler, Robert David; Tattersall, Rachel Scarlett; Schoemans, Helene; Greco, Raffaella; Badoglio, Manuela; Labopin, Myriam; Alexander, Tobias; Kirgizov, Kirill; Rovira, Montserrat; Saif, Muhammad; Saccardi, Riccardo; Delgado, Julio; Perić, Zinaida; Koenecke, Christian; Penack, Olaf; Basak, Grzegorz; Snowden, John Andrew More authors...

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Sandler, R. D., Tattersall, R. S., Schoemans, H., Greco, R., Badoglio, M., Labopin, M. ... Snowden, J. A. (2020). Diagnosis and Management of Secondary HLH/MAS Following HSCT and CAR-T Cell Therapy in Adults; A Review of the Literature and a Survey of Practice Within EBMT Centres on Behalf of the Autoimmune Diseases Working Party (ADWP) and Transplant Complications Working Party (TCWP). Frontiers in Immunology, 11.. doi: 10.3389/fimmu.2020.00524

Sandler, Robert David, et al. "Diagnosis and Management of Secondary HLH/MAS Following HSCT and CAR-T Cell Therapy in Adults; A Review of the Literature and a Survey of Practice Within EBMT Centres on Behalf of the Autoimmune Diseases Working Party (ADWP) and Transplant Complications Working Party (TCWP)." Frontiers in Immunology, vol. 11, 2020. https://doi.org/10.3389/fimmu.2020.00524

Sandler, Robert David, Rachel Scarlett Tattersall, Helene Schoemans, Raffaella Greco, Manuela Badoglio, Myriam Labopin, Tobias Alexander, et al. "Diagnosis and Management of Secondary HLH/MAS Following HSCT and CAR-T Cell Therapy in Adults; A Review of the Literature and a Survey of Practice Within EBMT Centres on Behalf of the Autoimmune Diseases Working Party (ADWP) and Transplant Complications Working Party (TCWP)." Frontiers in Immunology 11 (2020). https://doi.org/10.3389/fimmu.2020.00524

Sandler, R. D., et al. (2020) 'Diagnosis and Management of Secondary HLH/MAS Following HSCT and CAR-T Cell Therapy in Adults; A Review of the Literature and a Survey of Practice Within EBMT Centres on Behalf of the Autoimmune Diseases Working Party (ADWP) and Transplant Complications Working Party (TCWP)', Frontiers in Immunology, 11. doi: 10.3389/fimmu.2020.00524

Sandler RD, Tattersall RS, Schoemans H, Greco R, Badoglio M, Labopin M, and sur.. Diagnosis and Management of Secondary HLH/MAS Following HSCT and CAR-T Cell Therapy in Adults; A Review of the Literature and a Survey of Practice Within EBMT Centres on Behalf of the Autoimmune Diseases Working Party (ADWP) and Transplant Complications Working Party (TCWP). Frontiers in Immunology [Internet]. 2020 March 31 [cited 2024 April 12];11. doi: 10.3389/fimmu.2020.00524

R. D. Sandler, et al., "Diagnosis and Management of Secondary HLH/MAS Following HSCT and CAR-T Cell Therapy in Adults; A Review of the Literature and a Survey of Practice Within EBMT Centres on Behalf of the Autoimmune Diseases Working Party (ADWP) and Transplant Complications Working Party (TCWP)", Frontiers in Immunology, vol. 11, March 2020. [Online]. Available at: https://urn.nsk.hr/urn:nbn:hr:105:548202. [Accessed: 12 April 2024]

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Title (english)Diagnosis and Management of Secondary HLH/MAS Following HSCT and CAR-T Cell Therapy in Adults; A Review of the Literature and a Survey of Practice Within EBMT Centres on Behalf of the Autoimmune Diseases Working Party (ADWP) and Transplant Complications Working Party (TCWP)
AuthorRobert David Sandler
AuthorRachel Scarlett Tattersall
AuthorHelene Schoemans
AuthorRaffaella Greco
AuthorManuela Badoglio
AuthorMyriam Labopin
AuthorTobias Alexander
AuthorKirill Kirgizov
AuthorMontserrat Rovira
AuthorMuhammad Saif
AuthorRiccardo Saccardi
AuthorJulio Delgado
AuthorZinaida Perić
AuthorChristian Koenecke
AuthorOlaf Penack
AuthorGrzegorz Basak
AuthorJohn Andrew Snowden
Author's institutionUniversity of Zagreb
School of Medicine
(Department of Internal Medicine)
Scientific / art field,
discipline and subdiscipline		BIOMEDICINE AND HEALTHCARE
Clinical Medical Sciences
Internal Medicine
Abstract (english)
Introduction: Secondary haemophagocytic lymphohistiocytosis (sHLH) or Macrophage Activation Syndrome (MAS) is a life-threatening hyperinflammatory syndrome that can occur in patients with severe infections, malignancy or autoimmune diseases. It is also a rare complication of haematopoetic stem cell transplantation (HSCT), with a high mortality. It may be associated with graft vs. host disease in the allogeneic HSCT setting. It is also reported following CAR-T cell therapy, but differentiation from cytokine release syndrome (CRS) is challenging. Here, we summarise the literature and present results of a survey of current awareness and practice in EBMT-affiliated centres of sHLH/MAS following HSCT and CAR-T cell therapy. ----- Methods: An online questionnaire was sent to the principal investigators of all EBMT member transplant centres treating adult patients (18 years and over) inviting them to provide information regarding: number of cases of sHLH/MAS seen in their centre over 3 years (2016-2018 inclusive); screening strategies and use of existing diagnostic/classification criteria and treatment protocols. ----- Results: 114/472 centres from 24 different countries responded (24%). We report estimated rates of sHLH/MAS of 1.09% (95% CI = 0.89-1.30) following allogeneic HSCT, 0.15% (95% CI = 0.09-5.89) following autologous HSCT and 3.48% (95% CI = 0.95-6.01) following CAR-T cell therapy. A majority of centres (70%) did not use a standard screening protocol. Serum ferritin was the most commonly used screening marker at 78% of centres, followed by soluble IL-2 receptor (24%), triglycerides (15%), and fibrinogen (11%). There was significant variation in definition of "clinically significant" serum ferritin levels ranging from 500 to 10,000 μg/mL. The most commonly used criteria to support diagnosis were HLH-2004 (43%) and the H score (15%). Eighty percent of responders reported using no standard management protocol, but reported using combinations of corticosteroids, chemotherapeutic agents, cytokine blockade, and monoclonal antibodies. ----- Conclusions: There is a remarkable lack of consistency between EBMT centres in the approach to screening, diagnosis and management. Further research in this field is needed to raise awareness of and inform harmonised, evidence-based approaches to the recognition and treatment of sHLH/MAS following HSCT/CAR-T cell therapy.
Keywords (english)
Languageenglish
Publication typeScientific paper - Original scientific paper
Publication statusPublished
Peer reviewPeer review
Publication versionPublished version
Journal titleFrontiers in Immunology
Numberingvol. 11, 524
e-ISSN1664-3224
DOIhttps://doi.org/10.3389/fimmu.2020.00524
URN:NBNurn:nbn:hr:105:548202
Publication2020-03-31
Document URLhttps://www.frontiersin.org/article/10.3389/fimmu.2020.00524/full
Type of resourceText
Access conditionsOpen access
Terms of useLicence Attribution 4.0 International (CC BY 4.0) icon
Created on2021-04-15 13:26:07