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.
MeSH: 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, Polyethylene Glycols, administration & dosage, Pyrazoles, administration & dosage, Treatment Outcome