A 71-year-old man had a 3-year history of a recurrent seronegative autoimmune constrictive pericarditis and pleural effusions, which were treated with pericardiectomy and prednisone and azathioprine. The pleural fluid was transudative and sterile, and the pericardium contained focal lymphoplasmacytic aggregates. Attempts to taper immunosuppressive drugs failed because of a recrudescence of the inflammatory syndrome and pericardial effusion. The patient was admitted with fever of unknown origin with a surge of the inflammatory syndrome and pancytopenia. He reported night sweats beginning 3 weeks prior associated with fever and unintentional weight loss. No lymphadenopathy was noted.Laboratory evaluations demonstrated a C-reactive protein level of 133 mg/L (reference range, <10 mg/L); ferritin level of 2607 ng/mL (reference, 30-300 ng/mL); lactate dehydrogenase (LDH) level of 289 U/L (reference, 135-214 U/L); pancytopenia with a hemoglobin level of 9.3 g/dL (reference, 13.3-17.7 g/dL), white blood cell count of 2700/μL (reference, 4000-10 000/μL), and platelet count of 99 ×10 3 /μL (reference, 150-350 ×10 3 /μL); and prerenal acute kidney injury (Acute Kidney Injury Network stage 1) with a serum creatinine level of 1.4 mg/dL (reference, 0.7-1.2 mg/dL). Results of a comprehensive serologic workup were negative. Gastroscopy results showed mycotic distal esophagitis.The patient was readmitted 3 weeks later with fever and hypotension without improvement despite broad-spectrum antibiotics and antifungals. Considering the increase in ferritin and LDH levels and pancytopenia, a hematologic disease was suspected. The patient did not report any bone pain. Computed tomography (CT) results showed a splenomegaly without evidence of metastatic disease or lymph node enlargement. Positron emission tomography (PET)-CT imaging (Figure, A and B) and a bone-marrow biopsy (Figure, C and D) were performed. A rapidly evolving sepsislike syndrome led to the patient's death.
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