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Empiric doxycycline that he was on was discontinued following negative initial rickettsial serology based on the non\u2010endemicity of Rocky Mountain spotted fever (RMSF) in Northeast Ohio. He demonstrated high inflammatory markers and met the criteria for hemophagocytic lymphohistiocytosis (HLH). With a working diagnosis of macrophage activation syndrome secondary to presumed systemic\u2010onset juvenile idiopathic arthritis (soJIA), he received HLH\u2010directed therapy. Rising antibody titers in convalescent sera established the diagnosis of RMSF. The patient recovered completely with HLH directed therapy and re\u2010institution of doxycycline. This is the first pediatric case report of <jats:italic>Rickettsia rickettsii<\/jats:italic> induced HLH demonstrating a favorable outcome despite modified therapy.<\/jats:p>","DOI":"10.1002\/jha2.405","type":"journal-article","created":{"date-parts":[[2022,4,5]],"date-time":"2022-04-05T09:11:39Z","timestamp":1649149899000},"page":"463-466","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Secondary hemophagocytic lymphohistiocytosis associated with Rocky Mountain spotted fever in a toddler: A case report"],"prefix":"10.1002","volume":"3","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5374-5888","authenticated-orcid":false,"given":"Smitha","family":"Hosahalli Vasanna","sequence":"first","affiliation":[{"name":"Department of Pediatric Hematology\u2010Oncology Rainbow Babies and Children's Hospital\/ University Hospitals  Cleveland Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter Paul C.","family":"Lim","sequence":"additional","affiliation":[{"name":"Department of Pediatric Infectious Diseases Rainbow Babies and Children's Hospital\/University Hospitals  Cleveland Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tanya Saeeda","family":"Khan","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Rainbow Babies and Childreny's Hospital\/University Hospitals  Cleveland Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jignesh","family":"Dalal","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology\u2010Oncology Rainbow Babies and Children's Hospital\/ University Hospitals  Cleveland Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,4,5]]},"reference":[{"issue":"2","key":"e_1_2_8_2_1","first-page":"1","article-title":"Morbidity and Mortality Weekly Report (MMWR) A Practical Guide for Health Care and Public Health Professionals","volume":"65","author":"Biggs HM","year":"2019","journal-title":"ejHaem"},{"key":"e_1_2_8_3_1","unstructured":"Hhs.gov. 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