[Case of tuberculosis-associated hemophagocytic syndrome in a hemodialysis patient under steroid therapy].

A 70-year-old woman was referred and admitted to our hospital with fever of unknown etiology. She had a past medical history of pulmonary tuberculosis. Ten weeks before admission she was diagnosed with acute renal failure caused by crescentic glomerulonephritis. Oral steroid therapy was not effective and she required dialysis. On admission, she was started on empiric antibiotic treatment, with the suspicion of bacterial infection. On the 3rd hospital day, she developed sudden hypotension and underwent direct hemoperfusion with a polymyxin B immobilized fiber. Soon after, her blood pressure normalised. Her inflammatory level apparently then improved in terms of white blood cell count and C-reactive protein, although severe fatigue and liver dysfunction persisted. On the 17th hospital day, her blood pressure went down again, accompanied by progressive pancytopenia and significant increase in serum vitamin B12, lactate dehydrogenase and uric acid. The patient was transmitted to the intensive care unit where she received bone marrow aspiration. The result revealed marked hemophagocytosis. Suspecting lymphoma-associated hemophagocytic syndrome (HPS), we administered high-dose steroid and combination chemotherapy. The treatment had no effect, and the patient died on the 21st hospital day. The autopsy demonstrated a large number of tuberculous bacilli, marked hemophagocytosis and necrosis without granuloma formation in multiple organs, leading to the pathological diagnosis of tuberculosis-associated HPS. Tuberculosis in one of the major causes for morbidity and mortality in hemodialyzed patients. It often shows atypical clinical manifestation and is difficult to diagnose. HPS in general runs a mild course unless it is lymphoma or EB virus-associated. This case seemed like bacterial infection improved with antibiotics but turned out to be a rapidly progressive tuberculosis-associated HPS. A careful examination and extensive laboratory workup is necessary to rule out tuberculosis, particularly in patients undergoing hemodialysis.