Steven-Johnson syndrome (SJS)- and toxic epidermal necrolysis (TEN)-like acute graft-vs-host disease (aGVHD) is associated with poor clinical outcomes and a high mortality rate. These findings were published in the Journal of the American Academy of Dermatology.
This multicenter, retrospective study sourced data from the largest medical health system in Taiwan that receives referrals for SJS/TEN. Patients (N=31) who developed aGHVD between 2000 and 2021 were evaluated for outcomes on the basis of developing SJS/TEN-like aGVHD or aGVHD alone.
A total of 15 patients developed SJS/TEN-like aGVHD and 16 did not. The SJS/TEN-like aGVHD and control cohorts comprised patients with a mean [SD] age of 38.7 [11.2] and 38.5 [10.5] years. In these cohorts, 53.3% and 62.5% of patients were men, 86.7% and 100% had a hematologic malignancy, 53.3% and 18.8% received a bone marrow transplant, 33.3% and 81.3% received a peripheral blood transplant, 46.7% and 35.7% received a transplant from an unrelated donor, and the median time from transplant to aGVHD onset was 27.5 (IQR, 15-52.5) and 70.5 (IQR, 25.5-172.5; P =.054) days, respectively.
In patients with SJS/TEN-like aGVHD, the mean [SD] total body surface area (TBSA) of skin detachment was 8.2% [10.1] and of erythema was 49.7% [20.2].
Patients with SJS/TEN-like aGVHD have a higher risk of experiencing leukopenia (odds ratio [OR], 19.500; P =.001), diarrhea (OR, 11.917; P =.004), liver dysfunction (OR, 10.833; P =.009), bacteremia (OR, 10.500; P =.009), severe dyskeratotic keratinocyte (OR, 10.000; P =.009), hepatitis (OR, 8.357; P =.023), pancytopenia (OR, 6.000; P =.032), and severe thrombocytopenia (OR, 6.000; P =.032) compared with patients without SJS/TEN-like aGVHD.
Patients with SJS/TEN-like aGVHD were at an increased risk for 5-year mortality compared with patients without SJS/TEN-like aGVHD (hazard ratio, 5.351; P <.001). Early mortality among the SJS/TEN-like aGVHD cohort were due to sepsis, multiorgan failure, and massive bleeding. Among patients without SJS/TEN-like aGVHD, there were no reports of early mortality.
The overall mortality rate throughout the follow-up was 80% for the SJS/TEN-like aGVHD and 25% for the non-SJS/TEN-like aGVHD groups (OR, 12.000; P <.0001). The time between developing SJS/TEN-like aGVHD and death ranged from 25 days to 40 months.
The major limitation of this study was the small sample size.
These data indicated that SJS/TEN-like aGVHD was associated with multiple systematic complications and a high risk for early and late mortality. Early differentiation of SJS/TEN-like aGVHD from drug induced-SJS/TEN and complications associated with aGVHD is necessary to prompt early intervention.
“[B]acteremia with sepsis was the main cause of death in SJS/TEN-like aGVHD. Infection surveillance of potential sources and pathogens with appropriate antibiotics is suggested in the presence of infection signs,” the researchers note. “Early recognition of SJS/TEN-like aGVHD, differential diagnoses from drug induced-SJS/TEN and other phenotypes of aGVHD, and prompt treatment initiation are necessary.”
Hung Y-T, Chen Y-W, Huang Y-L, Lin Y-J, Chen C-B, Chung W-H.. Acute graft-versus-host disease presenting as Stevens–Johnson syndrome and toxic epidermal necrolysis: a retrospective cohort study. J Am Acad Dermatol. 2022;S0190-9622(22)02961-9. doi:10.1016/j.jaad.2022.10.035