Hemophagocytic Lymphohistiocytosis: Early Treatment Leading to an Excellent Outcome

Affiliations

¹ is an Emergency Medicine-Critical Care Attending Physician, and is Chairperson Pulmonary-Critical Care Department, Pulmonary-Critical Care Fellowship Program Director, both at Veterans Affairs Caribbean Healthcare System in San Juan, Puerto Rico. is Critical Care Fellow at AdventHealth in Orlando, Florida.

Case Reports

Gerald Marín-García et al. Fed Pract. 2021 Aug.

. 2021 Aug;38(Suppl 3):e64-e66.

doi: 10.12788/fp.0162.

¹ is an Emergency Medicine-Critical Care Attending Physician, and is Chairperson Pulmonary-Critical Care Department, Pulmonary-Critical Care Fellowship Program Director, both at Veterans Affairs Caribbean Healthcare System in San Juan, Puerto Rico. is Critical Care Fellow at AdventHealth in Orlando, Florida.

Abstract

HLH is a rare and deadly disease increasingly more present in adults, but following treatment protocol may yield favorable results.

Author disclosures The authors report no actual or potential conflicts of interest with regard to this article.

1. Chen TY, Hsu MH, Kuo HC, Sheen JM, Cheng MC, Lin YJ. Outcome analysis of pediatric hemophagocytic lymphohistiocytosis. J Formos Med Assoc. 2021;120(1 pt 1):172–179. doi: 10.1016/j.jfma.2020.03.025. - DOI - PubMed
1. Henter JI, Samuelsson-Horne A, Aricò M, et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood. 2002;100(7):2367–2373. doi: 10.1182/blood-2002-01-0172. - DOI - PubMed
1. Henter JI, Elinder G, Ost A. Diagnostic guidelines for hemophagocytic lymphohistiocytosis. The FHL Study Group of the Histiocyte Society. Semin Oncol. 1991;18(1):29–33. - PubMed
1. Henter JI, Horne A, Aricó M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48(2):124–131. doi: 10.1002/pbc.21039. - DOI - PubMed
1. Knaak C, Nyvlt P, Schuster FS, et al. Hemophagocytic lymphohistiocytosis in critically ill patients: diagnostic reliability of HLH-2004 criteria and HScore. Crit Care. 2020;24(1):244. doi: 10.1186/s13054-020-02941-3. Published 2020 May 24. - DOI - PMC - PubMed