BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare disorder with rapid progression and high mortality.HLH occurs mostly due to infection,malignant tumors,and immune disorders.Among infections that cause HLH,viral infections,especially Epstein-Barr virus infections,are common,whereas tuberculosis is rare.Tuberculosis-associated HLH has a wide range of serological and clinical manifestations that are similar to those of systemic lupus erythematosus(SLE).CASE SUMMARY This study describes a case of tuberculosis-associated HLH misdiagnosed as SLE because of antinuclear antibody(ANA),Smith(Sm)antibody and lupus anticoagulant positivity;leukopenia;thrombocytopenia;pleural effusion;decreased C3,quantitatively increased 24 h urinary protein and fever.The patient was initially treated with glucocorticoids,which resulted in peripheral blood cytopenia and symptom recurrence.Then,caseating granulomas and hemophagocytosis were observed in her bone marrow.She was successfully treated with conventional category 1 antituberculous drugs.In addition,we reviewed the literature on tuberculosis-associated HLH documented in Pub Med,including all full-text articles published in English from December 2009 to December 2019,and summarized the key points,including the epidemiology,clinical manifestations,diagnosis,and treatment of tuberculosis-associated HLH and the differences of the present case from previous reports.CONCLUSION Tuberculosis should be considered in patients with fever or respiratory symptoms.Antituberculous drugs are important for treating tuberculosisassociated HLH.
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