Macrophage activation syndrome resistant to medical therapy in a patient with systemic lupus erythematosus and its remission with splenectomy.
Macrophage activation syndrome (MAS) is a rare, but potentially life-threatening complication of systemic lupus erythematosus (SLE). A bone marrow biopsy often provides pathologic evidence of MAS, and MAS usually responds to corticosteroids alone or with the addition of cyclosporine A. We describe a case of MAS developing in a pregnant patient with SLE, who presented with fever and pancytopenia. Extensive investigations could not find the evidence of infection. Although intensive medical treatment was performed with a suspicion of MAS based on clinical grounds, no response was observed and bone marrow biopsy showed no evidence of hemophagocytosis. Positron emission tomography/computed tomography (PET/CT) suggested the possible cause of fever was in the spleen where fluorodeoxyglucose uptake was markedly increased. After splenectomy, the patient was improved and numerous hemophagocytic macrophages were proved in the splenic tissue. With this unique case, we would like to emphasize that bone marrow biopsy cannot always be relied on in making a diagnosis of MAS and PET/CT can provide helpful information in the diagnosis of MAS.
Department of Internal Medicine, The Catholic University of Korea, Seoul, South Korea.
Ji Min
JM
Kim
Seung Ki
SK
Kwok
Ji Hyeon
JH
Ju
Kyung Su
KS
Park
Gyeong Sin
GS
Park
Ho Youn
HY
Kim
Sung Hwan
SH
Park
D000328:Adult; D005260:Female; D019788:Fluorodeoxyglucose F18; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D055501:Macrophage Activation Syndrome / Q000473:pathology / Q000601:surgery*; D064847:Multimodal Imaging; D049268:Positron-Emission Tomography; D011247:Pregnancy; D011248:Pregnancy Complications / Q000601:surgery*; D013156:Splenectomy; D014057:Tomography, X-Ray Computed
D019788:Fluorodeoxyglucose F18