Clinical outcomes of adults with hemophagocytic lymphohistiocytosis treated with the HLH-04 protocol: a retrospective analysis

Rachel J. Bubik, Dylan M. Barth, Christopher Hook, Robert C. Wolf, Jessica M. Muth, Kristin Mara, Mrinal S. Patnaik, Rajiv K. Pruthi, Ariela L. Marshall, Mark R. Litzow, Michelle A. Elliott, William J. Hogan, Mithun V. Shah, Kebede H. Begna, Hassan Alkhateeb, Animesh Pardanani, Aneel A. Ashrani, Timothy G. Call, Candido E. Rivera, John K. CamorianoRonald S. Go, Alexandra P. Wolanskyj-Spinner, Sameer A. Parikh

Research output: Contribution to journalArticle

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of pathologic immune activation in children that is increasingly being recognized in adults. Efficacy data for the HLH-04 protocol in adults is lacking. This study retrospectively analyzed 31 adult patients, median age 46 years, who received HLH-04 from 1/1/2004 to 5/1/2018. HLH etiology included malignancy (n = 9), autoimmune (n = 8), infection (n = 8), and idiopathic (n = 6). Eighteen patients were evaluable for response at week 4 with 7 having no response, 11 reaching partial response, and 0 reaching complete response (CR). Six patients eventually achieved CR at a median 195 days. The 1-year overall survival (OS) was 35% and median OS was 3.2 months. Univariate analysis showed shorter survival for hemoglobin <9 g/dL (HR 4.29, p = 0.003), platelets <100 × 109/L (HR 4.06, p = 0.027), ANC <1 × 109/L (HR 5.24, p = 0.001), and total bilirubin >1.2 mg/dL (HR 3.30, p = 0.022). Outcomes of adults treated with HLH-04 remain dismal and newer treatment modalities are needed.

Original languageEnglish (US)
Pages (from-to)1592-1600
Number of pages9
JournalLeukemia and Lymphoma
Volume61
Issue number7
DOIs
StatePublished - Jun 6 2020

Keywords

  • Hemophagocytic lymphohistiocytosis (HLH)
  • corticosteroids
  • etoposide
  • immune activation

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

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