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

Leukemia & Lymphoma
Rachel J BubikSameer A Parikh

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.

References

Apr 1, 1991·Acta paediatrica Scandinavica·J I HenterA Ost
Jun 1, 1983·European Journal of Pediatrics·G E Janka
Apr 30, 1998·Hematology/oncology Clinics of North America·G JankaJ I Henter
Aug 29, 2006·Pediatric Blood & Cancer·Jan-Inge HenterGritta Janka
Oct 5, 2007·The Oncologist·Diana SuperfinAngela M Davies
Dec 17, 2009·Hematology·Alexandra H Filipovich
May 22, 2013·American Journal of Clinical Pathology·Flavia G N Rosado, Annette S Kim
Dec 3, 2013·Lancet·Manuel Ramos-CasalsXavier Bosch
Dec 10, 2013·Hematology·Gritta E Janka, Kai Lehmberg
Mar 4, 2014·Mayo Clinic Proceedings·Sameer A ParikhAlexandra P Wolanskyj
May 20, 2014·The American Journal of Medicine·Sébastien RivièreLaurence Fardet
Jun 27, 2014·Journal of Blood Medicine·Melissa R George
Mar 12, 2015·Blood·Alison M Schram, Nancy Berliner
Oct 16, 2015·Hematology/oncology Clinics of North America·Alexandra H Filipovich, Shanmuganathan Chandrakasan
Dec 8, 2015·Hematology·Carl E Allen, Kenneth L McClain
Sep 19, 2018·Mayo Clinic Proceedings. Innovations, Quality & Outcomes·Pilar Brito-ZerónUNKNOWN REGHEM-GEAS-SEMI Study Group

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Citations

Apr 22, 2021·American Journal of Clinical Oncology·Wei YangXiaofeng Xu
Aug 5, 2021·Orphanet Journal of Rare Diseases·Shuyan YaoZhao Wang
Oct 26, 2021·Frontiers in Medicine·Amandine BichonJulien Carvelli

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