Refractory Macrophage Activation Syndrome in a Patient with Systemic Lupus Erythematosus Treated with Tocilizumab.
10.3904/kjm.2015.89.3.372
- Author:
Jin Sun OH
1
;
Jinoh PARK
;
Nah Kyum LEE
;
Seung Hyeon BAE
;
Yong Gil KIM
;
Chang Keun LEE
;
Bin YOO
Author Information
1. Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. bestmd2000@amc.seoul.kr
- Publication Type:Case Report
- Keywords:
Lymphohistiocytosis, Hemophagocytic;
Macrophage activation syndrome;
Lupus erythematosus, Systemic
- MeSH:
Adrenal Cortex Hormones;
Autoimmune Diseases;
Bone Marrow;
Cyclosporine;
Ferritins;
Fever;
Fever of Unknown Origin;
Hematologic Tests;
Histiocytes;
Humans;
Immunoglobulins;
Liver;
Lupus Erythematosus, Systemic*;
Lymphohistiocytosis, Hemophagocytic;
Macrophage Activation Syndrome*;
Macrophage Activation*;
Macrophages*;
Pancytopenia;
Rituximab
- From:Korean Journal of Medicine
2015;89(3):372-376
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Macrophage activation syndrome (MAS) is a secondary hemophagocytic lymphohistiocytosis caused by autoimmune diseases, such as systemic lupus erythematosus (SLE). It is characterized by fever, cytopenia, coagulopathy, hepatosplenomegaly, elevated liver enzyme, and high ferritin, typically combined with hemophagocytic histiocyte proliferation in the bone marrow. Here, we report a case of MAS in a patient with SLE treated successfully by tocilizumab. She was transferred to our hospital due to persistent fever of unknown origin. Initial blood tests revealed cytopenia, elevated liver enzyme, and high ferritin. Bone marrow histology revealed the presence of hemophagocytic histiocytes. The patient was initially treated with high dose corticosteroids; however, fever and cytopenia were not controlled. Additional treatments with cyclosporine, intravenous immunoglobulin, and rituximab were applied consecutively, but the fever and cytopenia persisted. Symptom resolution was finally achieved following treatment with tocilizumab, resulting in rapid improved of fever, and resolution of pancytopenia within 2 months.
