Etiologies and management of haemophagocytic lymphohistiocytosis: is it time for an updated protocol and targeted treatments?
Autor: | Posas-Mendoza TF; Department of Rheumatology, Ochsner Clinic Foundation, New Orleans, LA, USA., McLeod C; Department of Rheumatology, Ochsner Clinic Foundation, New Orleans, LA, USA., Davis W; Department of Rheumatology, Ochsner Clinic Foundation, New Orleans, LA, USA.; The University of Queensland, Faculty of Medicine, Ochsner Clinical School, New Orleans, LA, USA., Zakem J; Department of Rheumatology, Ochsner Clinic Foundation, New Orleans, LA, USA.; The University of Queensland, Faculty of Medicine, Ochsner Clinical School, New Orleans, LA, USA., Quinet R; Department of Rheumatology, Ochsner Clinic Foundation, New Orleans, LA, USA.; The University of Queensland, Faculty of Medicine, Ochsner Clinical School, New Orleans, LA, USA. |
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Jazyk: | angličtina |
Zdroj: | Rheumatology (Oxford, England) [Rheumatology (Oxford)] 2021 Jun 18; Vol. 60 (6), pp. 2927-2933. |
DOI: | 10.1093/rheumatology/keaa741 |
Abstrakt: | Objective: The objective of this study was to analyse the features, therapeutic approaches, and outcomes for adult patients with haemophagocytic lymphohistiocytosis (HLH) at a single centre. Methods: This study was a retrospective chart review of all patients >18 years of age diagnosed with HLH according to HLH-2004 or H-score criteria at Ochsner Medical Center-New Orleans between 2013 and 2019. Results: A total of 29 patients with HLH met inclusion criteria. A total of 7 patients had an underlying malignancy, 12 had an autoimmune disease, 2 were transplant patients, and 2 had a combination of malignancy, autoimmune disease, or immunodeficiency. A total of 6 patients developed HLH precipitated by infection alone. All 29 patients presented with fever. A total of 28 (97%) patients met H-score criteria, and only 20 (67%) met HLH-2004 criteria. Fifteen patients were treated with the HLH-2004 protocol. Of those treated with the HLH-2004 protocol, 73% (11/15) died, 8% (1/15) had recurrence of HLH, and 20% (3/15) had resolution of HLH. A total of 14 patients were treated with targeted therapy. Of those treated with targeted therapy, 93% (13/14) had resolution of HLH and 1 died. Targeted therapy included pulse steroids, tocilizumab, anakinra, IVIG, CSA, rituximab, and/or CYC in addition to antiviral or antibiotic therapy. Conclusion: Our findings suggested that the rheumatologic patient population responded well to a targeted therapeutic approach and poorly to the HLH-2004 protocol. Whether the poor outcomes found with the use of the HLH-2004 protocol are secondary to the protocol itself or the aggressive nature of malignancy-associated HLH is unclear. Further studies are needed to develop tailored therapeutic regimens. (© The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.) |
Databáze: | MEDLINE |
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