Open AccessRuxolitinib in conjunction with the HLH-94 protocol for Epstein-Barr virus-related hemophagocytic lymphohistiocytosis in the intensive care unit
Author(s) -
Zoufang Huang,
Jiangbo Xie
Publication year - 2021
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000025188
Subject(s) - medicine , hemophagocytic lymphohistiocytosis , pancytopenia , hepatosplenomegaly , cytopenia , sore throat , ruxolitinib , regimen , pediatrics , hypofibrinogenemia , immunology , myelofibrosis , bone marrow , disease , fibrinogen
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.