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.