M Zhou1,2, L Li1, Q Zhang3, S Ma1, J Sun1, L Zhu1, D Lu1, J Zhu1, D Zhou1, Y Zheng1, X Yang1, M Xie1, M Zhu1, X Ye1, W Xie1. 1. Senior Department of Hematology, The First Affiliated Hospital of Medical School of Zhejiang University, Hangzhou, Zhejiang, China. 2. Department of Hematology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang, China. 3. Department of Hematology, The 6th Affiliated Hospital of Wenzhou Medical University, People's Hospital of Lishui, Lishui, Zhejiang, China.
Abstract
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by an infrequent but immune-mediated life-threatening disease, with confusing clinical manifestations, rapidly deteriorating health, high morbidity and mortality and challenging diagnosis. AIM: The purpose of this study was to improve the recognition and understanding of HLH. DESIGN: Retrospective observational cross-sectional study. METHODS: Data were collected for all cases of adult patients diagnosed with HLH in a large cohort managed at a single medical center from January 2011 to December 2015. RESULTS: The median age was 52 years (range 18-90 years) and 123 (60.0%) were male. Over 95% patients manifested fever, hyperferritinemia and elevated lactate dehydrogenase. Underlying triggers of HLH were as follows: 119 (58.0%) malignancies, 83 (40.5%) infections, 14 (6.8%) unknown triggers and 14 (6.8%) autoimmune disorders. The median overall survival was 55 days. And elderly patients (age ≥60 years) had a markedly worse survival compared with young patients (age <60 years) (median overall survival 24 days vs. 159 days, respectively; P <0.001). In a multivariable analysis, platelet <40 × 109/l (HR = 2.534; 95% CI 1.152-5.573; P = 0.021), PT prolonged >3 s (HR = 1.909; 95% CI 1.127-3.234; P = 0.016) and malignancy (HR = 1.614; 95% CI 1.008-2.582; P = 0.046) were correlated with poor survival. CONCLUSION: HLH adult patients had very complex clinical manifestations as well as underlying diseases. Patients with PLT <40 ×109/l, PT prolonged >3 s and malignancy had inferior survival. It is of great importance to improve our understanding of this syndrome.
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by an infrequent but immune-mediated life-threatening disease, with confusing clinical manifestations, rapidly deteriorating health, high morbidity and mortality and challenging diagnosis. AIM: The purpose of this study was to improve the recognition and understanding of HLH. DESIGN: Retrospective observational cross-sectional study. METHODS: Data were collected for all cases of adult patients diagnosed with HLH in a large cohort managed at a single medical center from January 2011 to December 2015. RESULTS: The median age was 52 years (range 18-90 years) and 123 (60.0%) were male. Over 95% patients manifested fever, hyperferritinemia and elevated lactate dehydrogenase. Underlying triggers of HLH were as follows: 119 (58.0%) malignancies, 83 (40.5%) infections, 14 (6.8%) unknown triggers and 14 (6.8%) autoimmune disorders. The median overall survival was 55 days. And elderly patients (age ≥60 years) had a markedly worse survival compared with young patients (age <60 years) (median overall survival 24 days vs. 159 days, respectively; P <0.001). In a multivariable analysis, platelet <40 × 109/l (HR = 2.534; 95% CI 1.152-5.573; P = 0.021), PT prolonged >3 s (HR = 1.909; 95% CI 1.127-3.234; P = 0.016) and malignancy (HR = 1.614; 95% CI 1.008-2.582; P = 0.046) were correlated with poor survival. CONCLUSION: HLH adult patients had very complex clinical manifestations as well as underlying diseases. Patients with PLT <40 ×109/l, PT prolonged >3 s and malignancy had inferior survival. It is of great importance to improve our understanding of this syndrome.
Authors: Shipra Gandhi; Manu R Pandey; Elizabeth A Repasky; Marc S Ernstoff; Kristopher Attwood; Wenyan Ji; Agnieszka K Witkiewicz; Erik S Knudsen; Cheryl Allen; Joseph D Tario; Paul K Wallace; Carlos D Cedeno; Maria Levis; Suzanne Stack; Pauline Funchain; Joseph J Drabick; Mark J Bucsek; Igor Puzanov; Hemn Mohammadpour Journal: Clin Cancer Res Date: 2020-10-30 Impact factor: 13.801
Authors: Pilar Brito-Zerón; Belchin Kostov; Pedro Moral-Moral; Aleida Martínez-Zapico; Carmen Díaz-Pedroche; Guadalupe Fraile; Patricia Pérez-Guerrero; Eva Fonseca; Angel Robles; María P Vaquero-Herrero; María Andrés Calvo; María José Forner; Cesar Morcillo; José Larrañaga; Monica Rodriguez-Carballeira; Manuel Ruiz-Muñoz; Robert Hurtado-García; Sergio Prieto-González; Asun Aljibe Aguilar; Luis Caminal-Montero; Pilar Hernández-Jiménez; Cristina Rodríguez Fernández-Viagas; Pedro Castro; Victoria Morell Massó; Alejandra Flores-Chavez; Manuel Ramos-Casals Journal: Mayo Clin Proc Innov Qual Outcomes Date: 2018-08-01