Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation that occurs as either a familial disorder or as a sporadic condition in association with a variety of triggers. Infections are the most common cause of HLH in adults and should be searched for as early treatment usually results in a favorable outcome. Human monocytotropic ehrlichiosis (HME) is a very rare cause of HLH. Failure to consider ehrlichiosis can result in misdiagnosis and an increased length of hospitalization and healthcare cost as described in our report. Treatment for secondary HLH is aimed at reducing hypercytokinemia and eradicating inflammatory and infected cells. It is important to promptly initiate doxycycline when tick-borne diseases are being entertained as a possible trigger, as the antibiotic is effective, safe and inexpensive.
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