Case Presentation: We present a multicenter 3 case series of a rare, but important sepsis mimic.Case 1 72-year-old female presented with generalized abdominal pain. Labs showed acute renal failure, lactic acidosis, thrombocytopenia and markedly elevated LFTs. She was treated for presumed sepsis with broad spectrum antibiotics. She developed a coagulopathy, DIC, and Pancytopenia requiring blood products. Labs showed hyperferritinemia at 31000 ng/ml and LDH > 1000 U/L. Infectious work-up returned a positive Ehrlichia DNA. Case 269-year-old female with history of ANCA Vasculitis diagnosed in 2004 presented with fevers, chills, arthralgias, headache & cough for 1 week. Admitted with suspected sepsis and started on broad-spectrum antibiotics. Infectious work up including bacterial and viral was negative. Lab work-up was noted for elevated liver enzymes, thrombocytopenia, hyponatremia, AKI, elevated LDH, microscopic hematuria and proteinuria. She had worsening respiratory failure with bilateral pulmonary infiltrates. Acute relapse of ANCA vasculitis was suspected and was given pulse dose steroids. She required intubation for respiratory failure. Bronchoscopy was negative for alveolar hemorrhages. Labs showed elevated Ferritin levels of 54059 ng/ml, hypertriglyceridemia, hypofibrinogenemia. Case 340-year-old male with history of asthma, hypertension, and distant drug use presented after 1-week of body itching and AMS. Labs revealed acute anemia with hemoglobin of 6.3, thrombocytopenia, renal failure, hyperbilirubinemia, and elevated liver enzymes. CT imaging showed hepatomegaly, splenomegaly, and enlarged likely reactive retroperitoneal lymph nodes. Infectious process was suspected, broad spectrum antibiotics were initiated. Labs demonstrated LDH of 4086, hyperferritinemia of 22,900 ng/ml, positive DAT, undetectable haptoglobin, and coagulopathy with INR of 1.9 and fibrinogen of 127 mg/dl. Infectious work-up returned an elevated EBV DNA quant.
Discussion: The cases presented are consistent with a diagnosis of Hemophagocytic Lymphohistiocytosis (HLH) with varying triggers and clinical outcomes.All cases could be reasonably construed as severe sepsis with acute organ failure. Bone marrow biopsies were preformed and demonstrated hemophagocytosis in each case. In cases 2 & 3 soluble IL-2 receptor levels were also elevated at 10,219 and 31,470 U/ml respectively (RI 175 – 858). In 2/3 cases the patient’s condition deteriorated despite treatment (etoposide/dexamethasone) and the patients died. Infectious triggers were ultimately identified in 2/3 cases, EBV and Ehrilichiosis. The third was presumed to be autoimmune related.
Conclusions: Adult HLH is a rare disease with a 20-88% mortality rate. Familiarity is important for early diagnosis, due to nonspecific signs, symptoms and laboratory findings associated with HLH which can also be observed in sepsis syndromes, MODS and other cytokine storm syndromes.
