Acute graft-versus-host disease presenting as Stevens–Johnson syndrome and toxic epidermal necrolysis: A retrospective cohort study - 16/03/23
, Wen-Hung Chung, MD, PhD a, g, h, j, k, l, m, nAbstract |
Background |
Cutaneous manifestations resembling Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) have been reported in patients with acute graft-versus-host disease (aGVHD); however, the clinicopathological characteristics of SJS/TEN-like aGVHD remain unexplored.
Objective |
To investigate the clinicopathology, complications, and outcomes of patients with SJS/TEN-like aGVHD.
Methods |
We analyzed a multicenter cohort of patients with aGVHD between 2000 and 2021.
Results |
We analyzed 31 patients with aGVHD, including SJS/TEN-like (n = 15) and non-SJS/TEN-like (n = 16). Patients with SJS/TEN-like aGVHD had significantly more extensive erythema and skin detachment/mucositis. SJS/TEN-like aGVHD was significantly associated with higher aGVHD grading and systemic complications, including pancytopenia, leukopenia, anemia, severe thrombocytopenia, coagulation abnormality, hepatitis, diarrhea, renal dysfunction, and bacteremia. A significantly lower hemoglobin/red cell distribution width ratio was identified in SJS/TEN-like aGVHD. Histopathology showed significant severe dyskeratosis and interface change. Patients with SJS/TEN-like aGVHD had lower 2-month survival rates and 5.35-fold higher 5-year mortality rates than those with non-SJS/TEN-like aGVHD. Total mortality rates of patients with SJS/TEN-like aGVHD reached 80% during follow-up; sepsis predominated the causes of death.
Limitations |
Retrospective, nonrandomized study with a small sample size.
Conclusion |
SJS/TEN-like aGVHD is associated with multiple systemic complications and high mortality. Early recognition, differential diagnosis from drug-induced-SJS/TEN, and appropriate treatment are critical.
Le texte complet de cet article est disponible en PDF.Key words : acute graft-versus-host disease, complication, morbidity, mortality, predictor, prognosis, Stevens–Johnson syndrome, survival, toxic epidermal necrolysis
Abbreviations used : aGVHD, CMV, GVHD, IL, NK, OR, SCORTEN, SJS, TBSA, TEN, Tregs
Plan
| Drs Chen and Chung contributed equally to this work. |
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| Funding sources: This study was supported by research grants from the Ministry of Science and Technology, Taiwan (grant no. MOST 108-2314-B-182A-006-MY3, 111-2314-B-182A-111-MY3 to C.-B.C.; 110-2320-B-182A-014-MY3, 108-2314-B-182A-104-MY3, 110-2326-B-182A-003- to W.-H.C.) and Chang Gung Memorial Hospital, Taiwan (grant no. CMRPG3L0851 to C.-B.C.). |
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| IRB approval status: The study protocol was reviewed and approved by the Chang Gung Memorial Hospital (CGMH) Institutional Review Board (IRB) (CGMH IRB Approval Number: 202100778B0). |
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| The funder had no role in the design of the study, data collection, analysis, interpretation of data, or in writing the manuscript. |
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| Patient consent was obtained. |
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| Data availability statement: The datasets used and/or analyzed during the current study are available from the corresponding author (Chun-Bing Chen). |
Vol 88 - N° 4
P. 792-801 - avril 2023 Retour au numéro
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