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Am J Med. 2014 Nov;127(11):1118-25. doi: 10.1016/j.amjmed.2014.04.034. Epub 2014 May 14.

Reactive hemophagocytic syndrome in adults: a retrospective analysis of 162 patients.

Author information

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.

KEYWORDS:

Adult; Hemophagocytic lymphohistiocytosis; Hemophagocytic syndrome; Infection; Lymphoma; Outcome

PMID:
24835040
DOI:
10.1016/j.amjmed.2014.04.034
[Indexed for MEDLINE]

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