Journal Article

Prognostic Factors and Outcomes of Adults With Hemophagocytic Lymphohistiocytosis

Parikh, Sameer A., Wolanskyj, Alexandra P., Kumar, Shaji, Letendre, Louis, Kapoor, Prashant

2014

Overview

To describe the prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis (HLH), a rare disorder caused by pathologic activation of the immune system. The study population consisted of a consecutive cohort of adult (age ≥18 years) patients treated at Mayo Clinic in Rochester, Minnesota, from January 1, 1996, through December 31, 2011, in whom a diagnosis of HLH was suspected and subsequently confirmed by retrospective review using the HLH-04 diagnostic criteria. Of 250 adult patients suspected of having HLH, 62 met the HLH-04 diagnostic criteria and were included in the final analysis. The median age was 49 years (range, 18-87 years), and 42 (68%) were male. The underlying cause of HLH was malignant tumor in 32 patients (52%), infection in 21 patients (34%), autoimmune disorder in 5 patients (8%), and idiopathic disease in 4 patients (6%). After a median follow-up of 42 months, 41 patients (66%) had died. The median overall survival of the entire cohort was 2.1 months. The median overall survival of patients with tumor–associated HLH was 1.4 months compared with 22.8 months for patients with non-tumor–associated HLH (P=.01). The presence of a malignant tumor and hypoalbuminemia were significant predictors of inferior survival on multivariate analysis. In this large series of adults with secondary HLH treated at a single tertiary care center, patients with low serum albumin levels and tumor–associated HLH had a markedly worse survival. Hemophagocytic lymphohistiocytosis remains elusive and challenging to clinicians who must maintain a high index of suspicion. The recent discovery of several novel diagnostic and therapeutic modalities may improve outcomes of adult patients with HLH.

• Publisher: Elsevier Inc,Elsevier, Inc,Elsevier Limited
• Subject: Adolescent / Adult / Aged / Aged, 80 and over / Analysis / Antineoplastic Combined Chemotherapy Protocols - therapeutic use / B cells / Cause of Death / Cohort Studies / Databases, Factual / Disease-Free Survival / Female / Histiocytosis / Humans / Immune system / Internal Medicine / Kaplan-Meier Estimate / Lymphohistiocytosis, Hemophagocytic - diagnosis / Lymphohistiocytosis, Hemophagocytic - drug therapy / Lymphohistiocytosis, Hemophagocytic - genetics / Lymphohistiocytosis, Hemophagocytic - mortality / Male / Middle Aged / Oncogene Proteins - genetics / Patient outcomes / Prognosis / Proportional Hazards Models / Rare Diseases / Retrospective Studies / Risk Assessment / Severity of Illness Index / Survival Analysis / Young Adult