Case
50-year-old African American male with history of alcohol dependence, alcohol withdrawal and alcoholic hepatitis presented to emergency room with high grade fever, shortness of breath, productive purulent cough and hemoptysis. He also had abdominal pain, diarrhea, and back pain.   His labs were remarkable for pancytopenia (white blood cell count 1500/mcL, hemoglobin 7.8 g/dL, and platelets 95,000/mcL) and elevated liver enzymes (AST 249, ALT 131). Chest X-Ray was suggestive of pneumonia. He shortly progressed to respiratory failure and septic shock, requiring intubation, pressor support, and broad-spectrum antibiotics with vancomycin, and piperacillin-tazobactum. Patient was found to have necrotizing/cavitary Klebsiella pneumonia complicated with acute respiratory distress syndrome requiring prone positioning. A day later, platelet count trended down to 2000/mcL and was refractory to platelet transfusions. He also developed acute renal failure secondary to volume overload. At this time, hematology was consulted, and hematology performed bone marrow biopsy (as illustrated in figure 1) which revealed histiocytosis with impressive erythrophagocytosis. Diagnosis for HLH was made as it met 5 of the 8 criteria (fever +pancytopenia + ferritin >500 + triglycerides >250 + hemo-phagocytosis on bone marrow biopsy) invariably related with Klebsiella bacterial infection. He was started on etoposide, and dexamethasone 10 mg twice daily, intravenously. Within few days he also developed febrile neutropenia secondary to chemotherapy and HLH, thereafter antifungal (voriconazole), antiviral, and inhaled gentamicin were also added.  Hospital course was further complicated with necrosis of digits and toes bilaterally, following which he had to undergo right below knee, right thumb and index finger amputation. After sometime etoposide was stopped and dexamethasone was tapered.  After a total of 10 weeks in the hospital he was discharged home.