Sébastien Rivière1, Lionel Galicier2, Paul Coppo3, Christophe Marzac4, Cedric Aumont5, Olivier Lambotte6, Laurence Fardet7. 1. AP-HP, Hôpital Saint Antoine, Service de Médecine Interne, Paris, France; UPMC Université Paris 06, Faculté de Médecine Pierre et Marie Curie, Paris, France. Electronic address: sebastien.riviere@sat.aphp.fr. 2. AP-HP, Hôpital Saint Louis, Service d'Immunologie Clinique, Paris, France. 3. UPMC Université Paris 06, Faculté de Médecine Pierre et Marie Curie, Paris, France; AP-HP, Hôpital Saint Antoine, Service d'Hématologie, Paris, France. 4. AP-HP, Hôpital Saint Antoine, Service d'Hématologie Biologique, Paris, France. 5. AP-HP, Hôpital de Bicêtre, Service d'Hématologie Biologique, Paris, France. 6. AP-HP, Hôpital de Bicêtre, Service de Médecine Interne, Paris, France; Université Paris-Sud 11, Faculté de Médecine Paris-Sud 11, Le Kremlin Bicêtre, France. 7. AP-HP, Hôpital Saint Antoine, Service de Médecine Interne, Paris, France; UPMC Université Paris 06, Faculté de Médecine Pierre et Marie Curie, Paris, France.
Abstract
OBJECTIVES: Current knowledge in reactive hemophagocytic syndrome mainly relies on single-center case series including a relatively small number of patients. We aimed to identify a multicenter large cohort of adult patients with reactive hemophagocytic syndrome and to describe relevant clinical and laboratory features, underlying conditions, and outcome. METHODS: We conducted a multicenter study in 3 tertiary care centers in France over a 6-year period. The medical files of 312 patients with suspected hemophagocytic syndrome were retrospectively reviewed. Patients were classified with a positive, negative, or undetermined diagnosis of hemophagocytic syndrome by experts' consensus. RESULTS: Among the 312 patients fulfilling our inclusion criteria, 162 were classified with positive hemophagocytic syndrome (male, 67%; median age, 48 [35-62] years). Compared with patients without hemophagocytic syndrome, patients with hemophagocytic syndrome more frequently had an underlying immunodepression (45% vs 33%, P = .03) and exhibited higher temperature, ferritin, triglycerides, aspartate transaminase, bilirubin, lactate dehydrogenase, and C-reactive protein, and lower hemoglobin, leukocytes, platelets, and sodium levels. Only 70% of them had hemophagocytosis features on bone marrow aspiration. Hematologic malignancies, especially non-Hodgkin lymphomas, were the main trigger of hemophagocytic syndrome, accounting for 56% of cases. The early mortality rate (ie, within 1 month after diagnosis) was 20%. Patients with hematologic malignancies-associated hemophagocytic syndrome had a poorer early outcome than those with underlying infection. CONCLUSIONS: In this large, multicenter study, hematologic malignancies are the main disease associated with hemophagocytic syndrome in adults. Early mortality is high, and outcome is influenced by the underlying disease.
OBJECTIVES: Current knowledge in reactive hemophagocytic syndrome mainly relies on single-center case series including a relatively small number of patients. We aimed to identify a multicenter large cohort of adult patients with reactive hemophagocytic syndrome and to describe relevant clinical and laboratory features, underlying conditions, and outcome. METHODS: We conducted a multicenter study in 3 tertiary care centers in France over a 6-year period. The medical files of 312 patients with suspected hemophagocytic syndrome were retrospectively reviewed. Patients were classified with a positive, negative, or undetermined diagnosis of hemophagocytic syndrome by experts' consensus. RESULTS: Among the 312 patients fulfilling our inclusion criteria, 162 were classified with positive hemophagocytic syndrome (male, 67%; median age, 48 [35-62] years). Compared with patients without hemophagocytic syndrome, patients with hemophagocytic syndrome more frequently had an underlying immunodepression (45% vs 33%, P = .03) and exhibited higher temperature, ferritin, triglycerides, aspartate transaminase, bilirubin, lactate dehydrogenase, and C-reactive protein, and lower hemoglobin, leukocytes, platelets, and sodium levels. Only 70% of them had hemophagocytosis features on bone marrow aspiration. Hematologic malignancies, especially non-Hodgkin lymphomas, were the main trigger of hemophagocytic syndrome, accounting for 56% of cases. The early mortality rate (ie, within 1 month after diagnosis) was 20%. Patients with hematologic malignancies-associated hemophagocytic syndrome had a poorer early outcome than those with underlying infection. CONCLUSIONS: In this large, multicenter study, hematologic malignancies are the main disease associated with hemophagocytic syndrome in adults. Early mortality is high, and outcome is influenced by the underlying disease.
Authors: Kai Lehmberg; Kim E Nichols; Jan-Inge Henter; Michael Girschikofsky; Tatiana Greenwood; Michael Jordan; Ashish Kumar; Milen Minkov; Paul La Rosée; Sheila Weitzman Journal: Haematologica Date: 2015-08 Impact factor: 9.941
Authors: Asra Ahmed; Samuel A Merrill; Fares Alsawah; Paula Bockenstedt; Erica Campagnaro; Sumana Devata; Scott D Gitlin; Mark Kaminski; Alice Cusick; Tycel Phillips; Suman Sood; Moshe Talpaz; Albert Quiery; Philip S Boonstra; Ryan A Wilcox Journal: Lancet Haematol Date: 2019-09-16 Impact factor: 18.959
Authors: Naval Daver; Kenneth McClain; Carl E Allen; Sameer A Parikh; Zaher Otrock; Cristhiam Rojas-Hernandez; Boris Blechacz; Sa Wang; Milen Minkov; Michael B Jordan; Paul La Rosée; Hagop M Kantarjian Journal: Cancer Date: 2017-06-16 Impact factor: 6.860