Background/Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially life-threatening disease. Secondary HLH is associated with various clinical conditions, including infections, malignancies, and autoimmune diseases. Although many previous studies reviewed the clinical features, treatments, and outcomes of autoimmune- or infection-associated HLH, respectively, only a few studies have evaluated the differences between autoimmune- and infection-associated HLH to these factors. The purpose of our study was to identify and compare the clinical features, treatments, and outcomes of patients with secondary HLH caused by diseases other than malignancy at a single institution.

Methods: We retrospectively collected data on 33 adult patients who were diagnosed with autoimmune- or infection-associated HLH from 1997 to 2011 at a single tertiary hospital. Patients were eligible if they were over 15 years of age and met five criteria for HLH. Patients were classified as having autoimmune- or infection-associated HLH and the medical data on each patient were reviewed.

Results: Twelve patients were diagnosed as having autoimmune-associated HLH. Among the 12 patients, nine patients had SLE and three patients had adult-onset still’s disease. Steroid therapy was given to all patients. Eleven patients recovered from autoimmune associated HLH. Twenty one patients were diagnosed as having infection-associated HLH. The most common infection associated with HLH was EBV (n = 19, 90.5%) followed by hepatitis A virus (n = 1, 4.8%) and parvovirus B19 (n = 1, 4.8%). Thirteen patients were treated according to the HLH protocol. Among the 21 patients, five patients underwent allogenic HCT. Only four patients survived without disease; three of the five transplant recipients and one of the 16 patients who had received no transplants. With respect to clinical characteristics, splenomegaly was more common in patients with infection-associated HLH (p = 0.010). With respect to laboratory characteristics, the platelet count and the level of ESR were lower in the infection-associated HLH group (p = 0.009 and p = 0.020). Hyperbilirubinemia was more prominent in the infection-associated HLH group (p = 0.015). Concerning treatment, patients with infection-associated HLH received the more commonly administered cyclosporine A and etoposide therapy than patients with autoimmune-associated HLH. Patients with infection-associated HLH (19.0%) had a lower survival rate than patients with autoimmune-associated HLH (91.7%).

Conclusion: Secondary HLH is a syndrome-based diagnosis as it encompasses various heterogeneous conditions. In the present study, autoimmune-associated HLH has mild disease activity and is included mild disease entity. Although there is still considerable debate regarding the ideal initial management approach for patients with autoimmune-associated HLH, corticosteroid administration alone seems to be sufficient. In secondary HLH, an exhaustive search for an underlying cause, such as infection or autoimmune disease, is warranted as the results of such research may guide treatment regimens and help predict outcomes.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/a-clinical-analysis-of-adult-patients-with-autoimmune-and-infection-associated-hemophagocytic-lymphohistiocytosis/