Systemic Lupus Erythematosus Associated Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review
American Journal of Internal Medicine
Volume 2, Issue 3, May 2014, Pages: 49-53
Received: May 17, 2014;
Accepted: May 28, 2014;
Published: Jun. 10, 2014
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Po-Chung Cheng, Department of Internal Medicine, National Yang Ming University, Taipei, Taiwan, ROC
Yun-Chung Cheng, Department of Radiology, National Taiwan University, Taipei, Taiwan, ROC
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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.
Hemophagocytic Lymphohistiocytosis, Systemic Lupus Erythematosus, Autoimmune Disease
To cite this article
Systemic Lupus Erythematosus Associated Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review, American Journal of Internal Medicine.
Vol. 2, No. 3,
2014, pp. 49-53.
Freeman HR, Ramanan AV. Review of haemophagocytic lymphohistiocytosis. Arch Dis Child. 2011; 96(7):688-693.
Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Eur J Pediatr. 2007; 166(2): 95-109.
Filipovich A, McClain K, Grom A. Histiocytic Disorders: Recent Insights into Pathophysiology and Practical Guidelines. Biol Blood Marrow Transplant. 2010; 16(1 suppl): S82-S89.
Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lympho-histiocytosis. Pediatr Blood Cancer. 2007; 48(2): 124-131.
Lin TF, Ferlic-Stark LL, Allen CE, Kozinetz CA, McClain KL. Rate of decline of ferritin in patients with hemophagocytic lymphohisti-ocytosis as a prognostic variable for mortality. Pediatr Blood Cancer. 2001; 56: 154-155.
Fukaya S, Yasuda S, Hashimoto T, Oku K, Kataoka H, Horita T, et al. Clinical fea-tures of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases. Rheumatology (Oxford). 2008; 47 (11): 1686-1691.
Horne A, Trottestam H, Arico M, Egeler RM, Filipovich AH, Gadner H, et al. Frequency and spectrum of central nervous system involvement in 193 children with haemophagocytic lymphohistiocytosis. Br J Haematol. 2008; 140(3): 327-335.
Henter JI, Samuelsson-Horne A, Arico M, Egeler RM, Elinder G, Filipovich AH, et al. Treatment of Haemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood. 2002; 100 (7): 2367-2373.
Imashuku S. Clinical features and treatment strategies for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. Crit Rev Oncol Hematol. 2002. 44(3): 259-272.
Doyle T, Bhagani S, Cwynarski K. Haemophagocytic syndrome and HIV. Curr Opin Infect Dis. 2009. 22(1): 1-6.