Ruxolitinib in conjunction with the HLH-94 protocol for Epstein-Barr virus-related hemophagocytic lymphohistiocytosis in the intensive care unit: A case report.

Abstract

RATIONALE:

The HLH-94 protocol is a standard induction treatment for hemophagocytic lymphohistiocytosis. However, about 30% of patients may not respond. Ruxolitinib has been clinically proven to be an effective treatment for hemophagocytic lymphohistiocytosis (HLH).

PATIENT CONCERNS:

A previously healthy 14-year-old girl presented to the local hospital with a 4-day history of persistent fever and sore throat.

DIAGNOSIS:

Clinical and laboratory tests revealed fever >38.5°C, hepatosplenomegaly, pancytopenia, hypertriglyceridemia, hypofibrinogenemia, hyperferritinemia, and an elevated interleukin-2 receptor level.

INTERVENTION:

This patient was treated with ruxolitinib and the HLH-94 protocol.

OUTCOMES:

The patient's clinical and some laboratory indices improved. Unfortunately, vital signs such as respiratory function and consciousness did not improve.

LESSONS:

This case report highlights the effect of using ruxolitinib in conjunction with the HLH-94 protocol. However, safety evaluation of this regimen was not performed because critically ill patient died too fast.

• Authors: Zoufang Huang, Jiangbo Xie
• Journal: Medicine (Medicine (Baltimore)) Vol. 100 Issue 11 Pg. e25188 (Mar 19 2021) ISSN: 1536-5964 [Electronic] United States
• PMID: 33726009 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

Chemical References

• Nitriles
• Pyrazoles
• Pyrimidines
• liposomal doxorubicin
• Polyethylene Glycols
• Etoposide
• Dexamethasone
• Doxorubicin
• ruxolitinib
• Methylprednisolone

Topics

• Adolescent
• Clinical Protocols
• Dexamethasone (administration & dosage)
• Doxorubicin (administration & dosage, analogs & derivatives)
• Drug Therapy, Combination
• Epstein-Barr Virus Infections (complications, drug therapy)
• Etoposide (administration & dosage)
• Fatal Outcome
• Female
• Herpesvirus 4, Human
• Humans
• Induction Chemotherapy
• Intensive Care Units
• Lymphohistiocytosis, Hemophagocytic (drug therapy, virology)
• Methylprednisolone (administration & dosage)
• Nitriles
• Polyethylene Glycols (administration & dosage)
• Pyrazoles (administration & dosage)
• Pyrimidines
• Treatment Outcome