On-line First

J Rheum Dis

Published online June 12, 2023

© Korean College of Rheumatology

Successful treatment of hemophagocytic lymphohistiocytosis in a patient with systemic lupus erythematosus with ruxolitinib: A case report

Ji In Jung, M.D.1 , Ju Yeon Kim, M.D.1 , Mi Hyeon Kim, M.D.2 , Jin Kyun Park, M.D., Ph.D.1 , Eun Young Lee, M.D., Ph.D.1 , Eun Bong Lee, M.D., Ph.D.1 , Jun Won Park, M.D.1

1Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, 2Division of Rheumatology, Department of Internal Medicine, Hallym University Dongtan Sacred Heart Hospital, Hwaseong, Korea

Correspondence to : Jun Won Park, https://orcid.org/0000-0002-8624-2582
Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, 101 Daehakro, Jongno-gu, Seoul 03080, Korea. E-mail: mpersonality@gmail.com

Received: May 4, 2023; Revised: May 15, 2023; Accepted: May 16, 2023

This is an Open Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

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.

Keywords Hemophagocytic lymphohistiocytosis, Janus kinase inhibitors, Ruxolitinib

Article

On-line First

J Rheum Dis

Published online June 12, 2023

Copyright © Korean College of Rheumatology.

Successful treatment of hemophagocytic lymphohistiocytosis in a patient with systemic lupus erythematosus with ruxolitinib: A case report

Ji In Jung, M.D.1 , Ju Yeon Kim, M.D.1 , Mi Hyeon Kim, M.D.2 , Jin Kyun Park, M.D., Ph.D.1 , Eun Young Lee, M.D., Ph.D.1 , Eun Bong Lee, M.D., Ph.D.1 , Jun Won Park, M.D.1

1Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, 2Division of Rheumatology, Department of Internal Medicine, Hallym University Dongtan Sacred Heart Hospital, Hwaseong, Korea

Correspondence to:Jun Won Park, https://orcid.org/0000-0002-8624-2582
Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, 101 Daehakro, Jongno-gu, Seoul 03080, Korea. E-mail: mpersonality@gmail.com

Received: May 4, 2023; Revised: May 15, 2023; Accepted: May 16, 2023

This is an Open Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

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.

Keywords: Hemophagocytic lymphohistiocytosis, Janus kinase inhibitors, Ruxolitinib

JRD
Jan 01, 2024 Vol.31 No.1, pp. 1~63
COVER PICTURE
Characteristic findings in interstitial lung disease (ILD) detected by lung ultrasound. Lung ultrasound can reveal characteristic findings in ILD. However, the definitive diagnosis of ILD typically requires a combination of clinical assessment, imaging studies (such as high-resuloution computed tomography [HRCT]), and sometimes a lung biopsy. (A) Traction bronchiectasis and parenchymal changes of upper lung in HRCT (arrows). (B) Corresponding changes of lung ultrasound presented by B-lines (arrows). (J Rheum Dis 2024;31:3-14)

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