TY - JOUR
T1 - sCD25 as an independent adverse prognostic factor in adult patients with HLH
T2 - results of a multicenter retrospective study
AU - Wimmer, Thomas
AU - Mattes, Raphael
AU - Stemmler, Hans Joachim
AU - Hauck, Fabian
AU - Schulze-Koops, Hendrik
AU - Stecher, Stephanie Susanne
AU - Starck, Michael
AU - Wendtner, Clemens Martin
AU - Bojko, Peter
AU - Hentrich, Marcus
AU - Nickel, Katharina E.
AU - Götze, Katharina S.
AU - Bassermann, Florian
AU - von Bergwelt-Baildon, Michael
AU - Spiekermann, Karsten
N1 - Publisher Copyright:
© 2023 by The American Society of Hematology.
PY - 2023/3/14
Y1 - 2023/3/14
N2 - Hemophagocytic lymphohistiocytosis (HLH) is a rare but often fatal hyperinflammatory syndrome caused by an inborn or acquired error of immunity. In adults, the underlying immunodeficiency generally arises alongside severe infections, malignancies, autoimmune diseases, and immunosuppressive treatment. To analyze risk factors and outcome in adults, we conducted a multicenter retrospective study. A total of 62 adult (age ≥18 years) patients met at least one of the following inclusion criteria: (1) ≥5 of 8 HLH-2004 criteria, (2) HScore ≥ 200 plus 4 HLH-2004 criteria, or (3) mutation compatible with an HLH diagnosis. Most patients (65%) were male, and the median age at diagnosis was 53.5 years (range, 19-81 years). All patients were assigned to 4 etiologic subgroups based on their most likely HLH trigger. The survival probability of the 4 etiologic subgroups differed significantly (P = .004, log-rank test), with patients with an underlying malignancy having the worst clinical outcome (1-year survival probability of 21%). The parameters older age, malignant trigger, elevated serum levels of aspartate transferase, creatinine, international normalized ratio, lactate dehydrogenase, sCD25, and a low albumin level and platelet count at treatment initiation were significantly (P < .1) associated with worse overall survival in the univariate Cox regression model. In multivariate analysis, sCD25 remained the only significant prognostic factor (P = .005). Our results suggest that sCD25 could be a useful marker for the prognosis of patients with HLH that might help to stratify therapeutic interventions.
AB - Hemophagocytic lymphohistiocytosis (HLH) is a rare but often fatal hyperinflammatory syndrome caused by an inborn or acquired error of immunity. In adults, the underlying immunodeficiency generally arises alongside severe infections, malignancies, autoimmune diseases, and immunosuppressive treatment. To analyze risk factors and outcome in adults, we conducted a multicenter retrospective study. A total of 62 adult (age ≥18 years) patients met at least one of the following inclusion criteria: (1) ≥5 of 8 HLH-2004 criteria, (2) HScore ≥ 200 plus 4 HLH-2004 criteria, or (3) mutation compatible with an HLH diagnosis. Most patients (65%) were male, and the median age at diagnosis was 53.5 years (range, 19-81 years). All patients were assigned to 4 etiologic subgroups based on their most likely HLH trigger. The survival probability of the 4 etiologic subgroups differed significantly (P = .004, log-rank test), with patients with an underlying malignancy having the worst clinical outcome (1-year survival probability of 21%). The parameters older age, malignant trigger, elevated serum levels of aspartate transferase, creatinine, international normalized ratio, lactate dehydrogenase, sCD25, and a low albumin level and platelet count at treatment initiation were significantly (P < .1) associated with worse overall survival in the univariate Cox regression model. In multivariate analysis, sCD25 remained the only significant prognostic factor (P = .005). Our results suggest that sCD25 could be a useful marker for the prognosis of patients with HLH that might help to stratify therapeutic interventions.
UR - http://www.scopus.com/inward/record.url?scp=85150451448&partnerID=8YFLogxK
U2 - 10.1182/bloodadvances.2022007953
DO - 10.1182/bloodadvances.2022007953
M3 - Article
C2 - 35973195
AN - SCOPUS:85150451448
SN - 2473-9529
VL - 7
SP - 832
EP - 844
JO - Blood Advances
JF - Blood Advances
IS - 5
ER -