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A comprehensive analysis of Lymphoma‐associated haemophagocytic syndrome in a large French multicentre cohort detects some clues to improve prognosis
Author(s) -
Bigenwald Camille,
Fardet Laurence,
Coppo Paul,
Meignin Véronique,
Lazure Thierry,
Fabiani Bettina,
Kohn Milena,
Oksenhendler Eric,
Boutboul David,
Uzzan Mathieu,
Lambotte Olivier,
Galicier Lionel
Publication year - 2018
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.15506
Subject(s) - lymphoma , medicine , epidemiology , etoposide , confidence interval , hematology , mortality rate , pediatrics , oncology , chemotherapy
Summary Lymphoma‐associated haemophagocytic syndrome ( LAHS ) accounts for most cases of secondary haemophagocytic syndrome ( HS ) and has been extensively described in Asian populations. However, little is known about the epidemiology of LAHS in Western countries. We herein report a case series of 71 LAHS patients in which the lymphomas were mainly of the aggressive type. Diagnoses included non‐Hodgkin B cell lymphoma (46·5%) including human herpes virus 8‐associated non‐Hodgkin lymphoma (12·7%), T cell lymphoma (28·2%) and Hodgkin lymphoma (23·9%). An underlying immunodeficiency was described in 30 patients (42·3%). Early mortality within the 30 days following HS diagnosis was observed in 26·8% of cases. The overall survival was estimated at 45·7% [95% confidence interval, CI (35·4–59·0)] at 6 months, and 34·3% [95% CI (24·8–47·4)] at 2 years. Concurrent infection, age over 50 years, ethnicity and etoposide treatment were independently associated with mortality. While it appears that certain types of lymphomas were more prone to trigger HS , LAHS were not restricted to a few types of lymphoma. The overall prognosis was poor, with a particularly high rate of early mortality, highlighting the importance of both early recognition and choice of initial therapeutic management.