Intravenous Immunoglobulin Therapy for Secondary Hemophagocytic Lymphohistiocytosis : A Retrospective Study of 46 Patients

Bertrand Dunogué¹, Magdalena Gerin², Claire Larroche³, Catherine Montagnier-Petrissans⁴, Loïc Guillevin⁵ and Luc Mouthon⁵, ¹Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France, ²Internal Medicine, Hôpital Jean Verdier, Bondy, France, ³Internal Medicine, Hôpital Avicenne, Bobigny, France, ⁴Groupe d'Expert AP-HP, Paris, France, ⁵National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: Intravenous immunoglobulin (IVIG), macrophage activation syndrome, outcomes and treatment

Session Information

Title: Miscellaneous Rheumatic and Inflammatory Diseases

Session Type: Abstract Submissions (ACR)

Background/Purpose

Intravenous Immunoglobulins (IVIg) have been reported as giving good results in infectious, but also auto-immune related forms of hemophagocytic lymphohistiocytosis (HLH), but only in case reports and small retrospective studies.

The objective of this study was to evaluate the use of IVIg as a treatment of secondary HLH in a wide hospital-based population.

Methods

A multicenter retrospective study of all cases of secondary HLH treated with IVIg in Parisian hospitals between January 2000 and December 2006.

Results

Of the 162 IVIg-treated patients for declared secondary HLH, 46 met the HLH-2004 criteria (29 male (63%), median age 47.5 yr (min 15; max 87)). Thirty-three (72%) had a history of immunodepression (AIDS (15/46), hemopathy (12/46), systemic disease (5/46)). Causes of HLH were: infection (18/46, 39.1%), malignant hemopathy (20/46, 43.5%), systemic disease (3/46, 6.5%), or undetermined (5/46, 10.8%). IVIg were administered mostly at 2 g/kg (25/46, 52%), with a median delay of 2 days (-12; 253) after diagnosis. One adverse event to IVIg administration occurred (shock). Other therapies included: corticosteroids (32/46, 70%), etoposide (10/46, 21.7%), chemotherapy (9/20 of the hemopathy group), anti-infectious agents (41/46, 89%), red blood cell (38/46, 82.6%) and platelet (30/46, 65%) transfusions. Twenty-nine patients (63%) required intensive care (respectively 10 (55.5%), 14 (70%), and 4 (80%) in the infection, hemopathy, and undetermined groups). Twenty-five patients (54.3%) died of HLH (respectively 6 (33.3%), 13 (65%), 2 (66.6%), and 4 (80%) in the infection, hemopathy, systemic disease, and undetermined groups), with a median time to death of 12 days (1; 142). While long-term survival was better in the infection group, short-term survival (at 20 and 60 days) and evolution of cytopenias did not vary significantly among the different etiological groups.

Conclusion

Short-term evolution of IVIg-treated HLH patients seems to be equally severe in all etiological groups, despite IVIg treatment. The impact of IVIg treatment on lower long-term mortality in the infectious-related group is hard to establish, owing to a higher long-term mortality related to the underlying cause in the hemopathy group.

Disclosure:

B. Dunogué,
None;

M. Gerin,
None;

C. Larroche,
None;

C. Montagnier-Petrissans,
None;

L. Guillevin,
None;

L. Mouthon,
None.

« Back to 2014 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/intravenous-immunoglobulin-therapy-for-secondary-hemophagocytic-lymphohistiocytosis-a-retrospective-study-of-46-patients/