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.