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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 Više autora...

Institucijski repozitorij: Dr Med - digitalni repozitorij Medicinskog fakulteta Sveučilišta u Zagrebu

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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, i 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]. 31.03.2020. [pristupljeno 02.12.2024.];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, Ožujak 2020. [Online]. Dostupno na: https://urn.nsk.hr/urn:nbn:hr:105:548202. [Citirano: 02.12.2024.]

Za citiranje koristite ovu mrežnu adresu: https://urn.nsk.hr/urn:nbn:hr:105:548202
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Naslov (engleski)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)
AutorRobert David Sandler
AutorRachel Scarlett Tattersall
AutorHelene Schoemans
AutorRaffaella Greco
AutorManuela Badoglio
AutorMyriam Labopin
AutorTobias Alexander
AutorKirill Kirgizov
AutorMontserrat Rovira
AutorMuhammad Saif
AutorRiccardo Saccardi
AutorJulio Delgado
AutorZinaida Perić
AutorChristian Koenecke
AutorOlaf Penack
AutorGrzegorz Basak
AutorJohn Andrew Snowden
Autorova ustanovaSveučilište u Zagrebu
Medicinski fakultet
(KATEDRA ZA INTERNU MEDICINU)
Znanstveno / umjetničko
područje, polje i grana		BIOMEDICINA I ZDRAVSTVO
Kliničke medicinske znanosti
Interna medicina
Sažetak (engleski)
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.
Ključne riječi (engleski)
Jezikengleski
Vrsta publikacijeZnanstveni rad - Izvorni znanstveni rad
Status objaveObjavljen
Vrsta recenzijeRecenziran
Verzija publikacijeObjavljena verzija rada (izdavačev PDF)
Naslov časopisaFrontiers in Immunology
Brojčani podacivol. 11, 524
e-ISSN1664-3224
DOIhttps://doi.org/10.3389/fimmu.2020.00524
URN:NBNurn:nbn:hr:105:548202
Datum objave publikacije2020-03-31
URL dokumentahttps://www.frontiersin.org/article/10.3389/fimmu.2020.00524/full
Vrsta resursaTekst
Prava pristupaOtvoreni pristup
Uvjeti korištenjaIkona licence Imenovanje 4.0 međunarodna (CC BY 4.0)
Datum i vrijeme pohrane2021-04-15 13:26:07