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Abstract
Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome characterized by an overactive immune response. HLH - 94 protocol had been widely accepted, however, the mortality rate remains significan plus major affect due to chemotherapy. Ruxolitinib, an JAK1/JAK2 inhibitor, has shown promise as a targeted therapy for HLH.
Case report: The 1st case: 11 year old boy, EBV (+), genes PFR1, UNC13D, STX11, STXBP2 (-), relapsed at the 20th week of therapy, combining with ruxolitinib. He no longer had fever after 7 days, complete blood count recovered after 28 days; but he relapsed after discontinuous ruxolitinib 1 week and die at week 26th. The 2nd case: 26 months old boy, EBV (+), genes PFR1, UNC13D (-), he relapsed at at the 4th week with sepsis, and only receiving 5 days with ruxolitinib at mortality due to severe respiratory failure at week 6th. The 3rd case 3: 21 month old girl, she did not undergo genes test, EBV (+), she still had fever, bloody diarrhea after 1 weak of initial therapy, ruxolitinib were indicated. After 3 day, her fever stopped, at day 5 bloody diarrhea went away, complete blood count normal at day 14th, her clinical is stable up to now (week 15th), no without replased.
Conclusion: The patient used ruxolitinib at week 2nd achieved reponse better than the others. Because of different clinical presentation, the effect of ruxolitinib may not accurate. Futher research, larger clinical trials are necessary to optimize the use of ruxolitinib and improve outcomes for patients with HLH.
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| Published | 26-12-2024 | |
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| Issue | No. 91 (2023) | |
| Section | Case report | |
| DOI | 10.38103/jcmhch.91.7 | |
| Keywords | Hội chứng thực bào máu, tái phát hoặc kháng trị, ruxolitinib, điều trị đầu tay Hemophagocytic lymphohistiocytosis, relapsed or refractory, ruxolitinib, front - line therapy |
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