Ehrlichiosis? Check for Hemophagocytic Lymphohistiocytosis

First Author(s)

• 

  Victoria Novoselova

  Mercy Hospital Saint Louis
  Saint Louis, MO

  Disclosure information not submitted.

  Slides and Audio

Co-Author(s)

• NO

  Nikhil Organti, MD, FACP

  Intensivist
  Mercy Hospital St. Louis
  Saint Louis

  Disclosure information not submitted.
• AK

  Anup Katyal, MD

  MD
  Mercy Hospital Saint Louis, United States

  Disclosure information not submitted.
• FS

  Farid Sadaka, MD, MD

  Mercy Hospital St Louis
  Saint Louis, MO

  Disclosure information not submitted.

Title: Ehrlichiosis??... Check for Hemophagocytic Lymphohistiocytosis (HLH)

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare complication of ehrlichiosis. We present 2 cases of HLH secondary to Ehrlichiosis in adults.

Description: Case 1: 80 yo female patient with history of atrial fibrillation, hypertension, bioprosthetic aortic valve presented with fever, vomiting for 3 days. She had leukopenia and thrombocytopenia. CT scan revealed enteritis. Broad spectrum antibiotics, vasopressors were started for septic shock, she was intubated for respiratory failure. Cultures were negative. Ferritin level was elevated at 70,089, LDH >2,500, triglycerides 377. Empiric doxycycline and steroids were added, awaiting tick PCR panel. Patient's condition progressively declined and developed multi-system organ failure. Etoposide was offered, however, the family declined treatment and withdrew care, given poor prognosis and patient's wishes. Later, it was found that Ehrlichia PCR was positive, and the soluble IL2 receptor level was elevated. The patient expired prior to diagnosis of ehrlichiosis and confirmation of HLH.
Case 2: 70 yo male patient presented to the ED with fever, myalgia for 3 days. He had mild thrombocytopenia. The patient was discharged from the ED but continued to be febrile and was hospitalized with pancytopenia, transaminitis, renal failure 2 days later . Doxycycline was started empirically. Ehrlichia PCR was positive, bone marrow biopsy revealed hemophagocytosis, ferritin was elevated at 18665 and soluble IL2 receptor level was elevated. Treatment was initiated with dexamethasone, etoposide. His condition progressively worsened, he developed multi-system organ failure, encephalopathy, underwent lumbar puncture with intrathecal methotrexate. The patient later developed candidemia and endocarditis, transitioned to comfort care as per family wishes.

Discussion: According to the CDC, the average annual incidence of Ehrlichiosis was 3.2 cases per million. As per current literature, there are about 50 documented HLH cases secondary to Ehrlichiosis. Given the high mortality rate with HLH, it is imperative to recognize and treat HLH early in Ehrlichiosis. We suggest checking screening ferritin level in suspected cases of Ehrlichiosis would help in early recognition and initiation of treatment HLH.