Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of immune overstimulation. HLH commonly manifests as multiple organ failure without apparent physiological stress. Primary HLH relates to genetic defects, whereas secondary HLH is triggered by infection, malignancy, or autoimmune disease. An aberrant natural killer cell or absence of T lymphocyte apoptosis causes uncontrolled inflammation. Secondary HLH in patients with systemic lupus erythematosus (SLE) is uncommon with an estimated prevalence of 0.9%. This report describes a male with SLE associated secondary HLH. Patients affected by HLH require prompt management, but delayed diagnosis is common due to its variable presentation. An eight week induction therapy with dexamethasone, etoposide, and intrathecal methotrexate increases survival in primary HLH and virus-associated secondary HLH. However, no standardized regimen exists for autoimmune associated HLH. Some patients respond to steroid monotherapy, while refractory patients may require cyclosporin, cyclophosphamide, or tacrolimus.
| Published in | American Journal of Internal Medicine (Volume 2, Issue 3) |
| DOI | 10.11648/j.ajim.20140203.13 |
| Page(s) | 49-53 |
| Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
| Copyright |
Copyright © The Author(s), 2014. Published by Science Publishing Group |
Hemophagocytic Lymphohistiocytosis, Systemic Lupus Erythematosus, Autoimmune Disease
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APA Style
Po-Chung Cheng, Yun-Chung Cheng. (2014). Systemic Lupus Erythematosus Associated Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review. American Journal of Internal Medicine, 2(3), 49-53. https://doi.org/10.11648/j.ajim.20140203.13
ACS Style
Po-Chung Cheng; Yun-Chung Cheng. Systemic Lupus Erythematosus Associated Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review. Am. J. Intern. Med. 2014, 2(3), 49-53. doi: 10.11648/j.ajim.20140203.13
AMA Style
Po-Chung Cheng, Yun-Chung Cheng. Systemic Lupus Erythematosus Associated Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review. Am J Intern Med. 2014;2(3):49-53. doi: 10.11648/j.ajim.20140203.13
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Download@article{10.11648/j.ajim.20140203.13,
author = {Po-Chung Cheng and Yun-Chung Cheng},
title = {Systemic Lupus Erythematosus Associated Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review},
journal = {American Journal of Internal Medicine},
volume = {2},
number = {3},
pages = {49-53},
doi = {10.11648/j.ajim.20140203.13},
url = {https://doi.org/10.11648/j.ajim.20140203.13},
eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajim.20140203.13},
abstract = {Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of immune overstimulation. HLH commonly manifests as multiple organ failure without apparent physiological stress. Primary HLH relates to genetic defects, whereas secondary HLH is triggered by infection, malignancy, or autoimmune disease. An aberrant natural killer cell or absence of T lymphocyte apoptosis causes uncontrolled inflammation. Secondary HLH in patients with systemic lupus erythematosus (SLE) is uncommon with an estimated prevalence of 0.9%. This report describes a male with SLE associated secondary HLH. Patients affected by HLH require prompt management, but delayed diagnosis is common due to its variable presentation. An eight week induction therapy with dexamethasone, etoposide, and intrathecal methotrexate increases survival in primary HLH and virus-associated secondary HLH. However, no standardized regimen exists for autoimmune associated HLH. Some patients respond to steroid monotherapy, while refractory patients may require cyclosporin, cyclophosphamide, or tacrolimus.},
year = {2014}
}
TY - JOUR T1 - Systemic Lupus Erythematosus Associated Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review AU - Po-Chung Cheng AU - Yun-Chung Cheng Y1 - 2014/06/10 PY - 2014 N1 - https://doi.org/10.11648/j.ajim.20140203.13 DO - 10.11648/j.ajim.20140203.13 T2 - American Journal of Internal Medicine JF - American Journal of Internal Medicine JO - American Journal of Internal Medicine SP - 49 EP - 53 PB - Science Publishing Group SN - 2330-4324 UR - https://doi.org/10.11648/j.ajim.20140203.13 AB - Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of immune overstimulation. HLH commonly manifests as multiple organ failure without apparent physiological stress. Primary HLH relates to genetic defects, whereas secondary HLH is triggered by infection, malignancy, or autoimmune disease. An aberrant natural killer cell or absence of T lymphocyte apoptosis causes uncontrolled inflammation. Secondary HLH in patients with systemic lupus erythematosus (SLE) is uncommon with an estimated prevalence of 0.9%. This report describes a male with SLE associated secondary HLH. Patients affected by HLH require prompt management, but delayed diagnosis is common due to its variable presentation. An eight week induction therapy with dexamethasone, etoposide, and intrathecal methotrexate increases survival in primary HLH and virus-associated secondary HLH. However, no standardized regimen exists for autoimmune associated HLH. Some patients respond to steroid monotherapy, while refractory patients may require cyclosporin, cyclophosphamide, or tacrolimus. VL - 2 IS - 3 ER -
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