Treatment and efficacy
HLH
DISEASE PROGRESSION, DISEASE, DISEASE
The treatment regimen of the ruxolitinib dosage consisted of two sequential phases: general-dose treatment with ruxolitinib, followed by individualized intensive treatment with escalated-dose ruxolitinib. Initially, all patients received oral ruxolitinib at a starting dose of 10/15 mg twice daily. In certain cases, corticosteroid, intravenous immunoglobulin (IVIG), and/or continuous renal replacement therapy were administered with ruxolitinib, but no chemotherapy was given before or during ruxolitinib treatment. Disease response evaluations were conducted at day 3, 7, 14, and 28 after ruxolitinib treatment. For patients with unfavorable responses (such as no improvement after 3 days of treatment, disease improvement but not achieving a favorable response, or disease progression at any time during general-dose treatment), the ruxolitinib dosage was escalated as determined by the clinicians based on the patient’s condition. The rate of dose escalation and the maximum dosage were tailored to each patient’s needs.The efficacy evaluation of the treatment was mainly based on the criteria previously described in studies for HLH (