Successful treatment of hemophagocytic lymphohistiocytosis in a patient with systemic lupus erythematosus with ruxolitinib : a case report
Copyright © 2024 by The Korean College of Rheumatology. All rights reserved..
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hematological disorder characterized by uncontrolled activation of CD8+ T and natural killer cells, leading to a cytokine storm and severe organ dysfunction. Although secondary HLH related to autoimmune diseases usually demonstrates a good treatment response to immunosuppressive therapy for underlying conditions, there is no consensus regarding the treatment in case of unresponsiveness to the treatment. Herein, we present a case of HLH that was unresponsive to high-dose glucocorticoid and cyclosporine treatment in a patient with newly diagnosed systemic lupus erythematosus. The patient's clinical features and laboratory abnormalities rapidly improved with ruxolitinib, an oral Janus kinase 1 and 2 (JAK1/2) inhibitor. This result suggests that blocking JAK-STAT pathway may be a potential treatment option in patients with refractory HLH secondary to autoimmune diseases.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:31 |
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| Enthalten in: |
Journal of rheumatic diseases - 31(2024), 2 vom: 01. Apr., Seite 125-129 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Jung, Ji In [VerfasserIn] |
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| Links: |
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| Themen: |
Case Reports |
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| Anmerkungen: |
Date Revised 03.04.2024 published: Print-Electronic Citation Status PubMed-not-MEDLINE |
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| doi: |
10.4078/jrd.2023.0027 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM370492919 |
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| 245 | 1 | 0 | |a Successful treatment of hemophagocytic lymphohistiocytosis in a patient with systemic lupus erythematosus with ruxolitinib |b a case report |
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| 520 | |a Copyright © 2024 by The Korean College of Rheumatology. All rights reserved. | ||
| 520 | |a Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hematological disorder characterized by uncontrolled activation of CD8+ T and natural killer cells, leading to a cytokine storm and severe organ dysfunction. Although secondary HLH related to autoimmune diseases usually demonstrates a good treatment response to immunosuppressive therapy for underlying conditions, there is no consensus regarding the treatment in case of unresponsiveness to the treatment. Herein, we present a case of HLH that was unresponsive to high-dose glucocorticoid and cyclosporine treatment in a patient with newly diagnosed systemic lupus erythematosus. The patient's clinical features and laboratory abnormalities rapidly improved with ruxolitinib, an oral Janus kinase 1 and 2 (JAK1/2) inhibitor. This result suggests that blocking JAK-STAT pathway may be a potential treatment option in patients with refractory HLH secondary to autoimmune diseases | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Hemophagocytic lymphohistiocytosis | |
| 650 | 4 | |a Janus kinase inhibitors | |
| 650 | 4 | |a Ruxolitinib | |
| 700 | 1 | |a Kim, Ju Yeon |e verfasserin |4 aut | |
| 700 | 1 | |a Kim, Mi Hyeon |e verfasserin |4 aut | |
| 700 | 1 | |a Park, Jin Kyun |e verfasserin |4 aut | |
| 700 | 1 | |a Lee, Eun Young |e verfasserin |4 aut | |
| 700 | 1 | |a Lee, Eun Bong |e verfasserin |4 aut | |
| 700 | 1 | |a Park, Jun Won |e verfasserin |4 aut | |
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