Secondary haemophagocytic lymphohistiocytosis in a patient with new-onset systemic lupus erythematosus: the challenges of timely diagnosis and successful treatment.
| Autor: | Shao D; Medicine, Jacobi Medical Center, Bronx, New York, USA., Pena O; Medicine, Jacobi Medical Center, Bronx, New York, USA., Sekulic M; Department of Pathology and Cell Biology, Columbia University Medical Center, New York, New York, USA., Valdez Imbert R; Medicine, Jacobi Medical Center, Bronx, New York, USA., Vegivinti CTR; Medicine, Jacobi Medical Center, Bronx, New York, USA., Jim B; Medicine, Jacobi Medical Center, Bronx, New York, USA belindajim286@gmail.com. |
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| Jazyk: | angličtina |
| Zdroj: | BMJ case reports [BMJ Case Rep] 2023 Jul 10; Vol. 16 (7). Date of Electronic Publication: 2023 Jul 10. |
| DOI: | 10.1136/bcr-2022-252938 |
| Abstrakt: | Haemophagocytic lymphohistiocytosis (HLH) is an immune-mediated disease driven by abnormal macrophage activation and regulatory cell dysfunction. HLH can be primary due to genetic mutations or secondary due to infection, malignancy or autoimmune conditions. We describe a woman in her early 30s who developed HLH while being treated for newly diagnosed systemic lupus erythematosus (SLE) complicated by lupus nephritis as well as concomitant cytomegalovirus (CMV) reactivation from a dormant infection. The trigger for this secondary form of HLH may have been either aggressive SLE and/or CMV reactivation. Despite prompt treatment with immunosuppressive therapies for SLE consisting of high-dose corticosteroids, mycophenolate mofetil, tacrolimus, etoposide for HLH and ganciclovir for CMV infection, the patient developed multiorgan failure and passed away. We demonstrate the difficulty in identifying a specific cause for secondary HLH when multiple conditions are present (SLE and CMV) and the fact that, despite aggressive treatment for both conditions, the mortality for HLH remains high. Competing Interests: Competing interests: None declared. (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.) |
| Databáze: | MEDLINE |
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