Reactive hemophagocytic syndrome in adults: a retrospective analysis of 162 patients.
OBJECTIVE
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.
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.
Sébastien
S
Rivière
AP-HP, Hôpital Saint Louis, Service d'Immunologie Clinique, Paris, France.
Lionel
L
Galicier
UPMC Université Paris 06, Faculté de Médecine Pierre et Marie Curie, Paris, France; AP-HP, Hôpital Saint Antoine, Service d'Hématologie, Paris, France.
Paul
P
Coppo
AP-HP, Hôpital Saint Antoine, Service d'Hématologie Biologique, Paris, France.
Christophe
C
Marzac
AP-HP, Hôpital de Bicêtre, Service d'Hématologie Biologique, Paris, France.
Cedric
C
Aumont
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.
Olivier
O
Lambotte
AP-HP, Hôpital Saint Antoine, Service de Médecine Interne, Paris, France; UPMC Université Paris 06, Faculté de Médecine Pierre et Marie Curie, Paris, France.
Laurence
L
Fardet
D000328:Adult; D000368:Aged; D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use; D001219:Aspartate Aminotransferases / Q000097:blood; D001663:Bilirubin / Q000097:blood; D002097:C-Reactive Protein / Q000032:analysis; D005047:Etoposide / Q000627:therapeutic use; D005260:Female; D005293:Ferritins / Q000097:blood; D005334:Fever / Q000453:epidemiology; D005602:France / Q000453:epidemiology; D005938:Glucocorticoids / Q000627:therapeutic use; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D016867:Immunocompromised Host; D007770:L-Lactate Dehydrogenase / Q000097:blood; D007958:Leukocyte Count; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy / Q000453:epidemiology*; D008223:Lymphoma / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009102:Multiple Organ Failure / Q000453:epidemiology; D010976:Platelet Count; D012189:Retrospective Studies; D012964:Sodium / Q000097:blood; D014280:Triglycerides / Q000097:blood
D000972:Antineoplastic Agents, Phytogenic; D005938:Glucocorticoids; D006454:Hemoglobins; D014280:Triglycerides; D005047:Etoposide; D002097:C-Reactive Protein; D005293:Ferritins; D012964:Sodium; D007770:L-Lactate Dehydrogenase; D001219:Aspartate Aminotransferases; D001663:Bilirubin
Adult; Hemophagocytic lymphohistiocytosis; Hemophagocytic syndrome; Infection; Lymphoma; Outcome