Korean J Med.  2015 Sep;89(3):372-376. 10.3904/kjm.2015.89.3.372.

Refractory Macrophage Activation Syndrome in a Patient with Systemic Lupus Erythematosus Treated with Tocilizumab

• Oh JS
• Park J
• Lee NK
• Bae SH
• Kim YG
• Lee CK
• Yoo B

• KMID: 2269016
• DOI: http://doi.org/10.3904/kjm.2015.89.3.372

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.

Keyword

Lymphohistiocytosis, Hemophagocytic; Macrophage activation syndrome; Lupus erythematosus, Systemic

MeSH Terms

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
Adrenal Cortex Hormones
Cyclosporine
Ferritins
Immunoglobulins