{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T20:19:14Z","timestamp":1790194754637,"version":"4.1.0"},"reference-count":120,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2024,2,21]],"date-time":"2024-02-21T00:00:00Z","timestamp":1708473600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Infectious Disease Reports"],"abstract":"<jats:p>Hemophagocytic lymphohistiocytosis (HLH) secondary to tick-borne infections is a rare but potentially life-threatening syndrome. We performed a scoping review according to PRISMA guidelines to systematically analyze the existing literature on the topic. A total of 98 patients were included, with a mean age of 43.7 years, of which 64% were men. Most cases, 31%, were reported from the USA. Immunosuppression was present in 21.4%, with the most common cause being previous solid organ transplantation. Constitutional symptoms were the most common, observed in 83.7% of the patients, while fever was reported in 70.4% of cases. Sepsis was present in 27.6%. The most common laboratory abnormalities in this cohort were thrombocytopenia in 81.6% of patients, while anemia, leukopenia, and leukocytosis were observed in 75.5%, 55.1%, and 10.2%, respectively. Liver enzyme elevation was noted in 63.3% of cases. The H-score was analyzed in 64 patients, with the mean value being 209, and bone marrow analysis was performed in 61.2% of patients. Ehrlichia spp. was the main isolated agent associated with HLH in 45.9%, followed by Rickettsia spp. in 14.3% and Anaplasma phagocytophilum in 12.2%. Notably, no patient with Powassan virus infection or Lyme borreliosis developed HLH. The most common complications were acute kidney injury (AKI) in 35.7% of patients, shock with multiple organ dysfunction in 22.5%, encephalopathy\/seizure in 20.4%, respiratory failure in 16.3%, and cardiac complications in 7.1% of patients. Treatment included antibiotic therapy alone in 43.9%, while 5.1% of patients were treated with immunosuppressants alone. Treatment with both antibiotics and immunosuppressants was used in 51% of patients. Appropriate empiric antibiotics were used in 62.2%. In 43.9% of cases of HLH due to tick-borne disease, patients received only antimicrobial therapy, and 88.4% of those recovered completely without the need for immunosuppressive therapy. The mortality rate in our review was 16.3%, and patients who received inappropriate or delayed empiric therapy had a worse outcome. Hence, we suggest empiric antibiotic treatment in patients who are suspected of having HLH due to tick-borne disease or in whom diagnostic uncertainty persists due to diagnostic delay in order to minimize mortality.<\/jats:p>","DOI":"10.3390\/idr16020012","type":"journal-article","created":{"date-parts":[[2024,2,21]],"date-time":"2024-02-21T08:43:48Z","timestamp":1708505028000},"page":"154-169","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["Hemophagocytic Lymphohistiocytosis (HLH) in Patients with Tick-Borne Illness: A Scoping Review of 98 Cases"],"prefix":"10.3390","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3790-3951","authenticated-orcid":false,"given":"Dorde","family":"Jevtic","sequence":"first","affiliation":[{"name":"Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"},{"name":"Department of Medicine, NYC Health + Hospitals\/Elmhurst, New York, NY 11373, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-2729-4861","authenticated-orcid":false,"given":"Marilia Dagnon","family":"da Silva","sequence":"additional","affiliation":[{"name":"Municipal University of S\u00e3o Caetano do Sul\u2014USCS Bela Vista, Sao Paulo 01327-000, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto Busmail","family":"Haylock","sequence":"additional","affiliation":[{"name":"Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"},{"name":"Department of Medicine, NYC Health + Hospitals\/Elmhurst, New York, NY 11373, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles W.","family":"Nordstrom","sequence":"additional","affiliation":[{"name":"Department of Hospital Medicine, Mayo Clinic Health System, Eau Claire, WI 54703, USA"},{"name":"Mayo Clinic College of Medicine and Science, Rochester, MN 55902, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stevan","family":"Oluic","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Mayo Clinic Health System, Mankato, MN 56001, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7798-4026","authenticated-orcid":false,"given":"Nikola","family":"Pantic","sequence":"additional","affiliation":[{"name":"Clinic of Hematology, University Clinical Center of Serbia, 11000 Belgrade, Serbia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-7557-4854","authenticated-orcid":false,"given":"Milan","family":"Nikolajevic","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Belgrade, 11000 Belgrade, Serbia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-1147-3392","authenticated-orcid":false,"given":"Nikola","family":"Nikolajevic","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Belgrade, 11000 Belgrade, Serbia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-8871-1617","authenticated-orcid":false,"given":"Magdalena","family":"Kotseva","sequence":"additional","affiliation":[{"name":"Internal Medicine Residency, Franciscan Health, Olympia Fields, Chicago, IL 60461, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5312-8812","authenticated-orcid":false,"given":"Igor","family":"Dumic","sequence":"additional","affiliation":[{"name":"Department of Hospital Medicine, Mayo Clinic Health System, Eau Claire, WI 54703, USA"},{"name":"Mayo Clinic College of Medicine and Science, Rochester, MN 55902, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,2,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"507","DOI":"10.5858\/arpa.2020-0802-RA","article-title":"Hemophagocytic Lymphohistiocytosis","volume":"146","author":"Ponnatt","year":"2022","journal-title":"Arch. Pathol. Lab. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1002\/pbc.21039","article-title":"HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis","volume":"48","author":"Henter","year":"2007","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1002\/(SICI)1096-911X(199705)28:5<342::AID-MPO3>3.0.CO;2-H","article-title":"HLH-94: A Treatment Protocol for Hemophagocytic Lymphohistiocytosis","volume":"28","author":"Henter","year":"1997","journal-title":"Med. Pediatr. Oncol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"2465","DOI":"10.1182\/blood.2018894618","article-title":"Recommendations for the management of hemophagocytic lymphohistiocytosis in adults","volume":"133","author":"Horne","year":"2019","journal-title":"Blood"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1548","DOI":"10.1182\/blood-2014-10-602607","article-title":"Marked hyperferritinemia does not predict for HLH in the adult population","volume":"125","author":"Schram","year":"2015","journal-title":"Blood"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.1182\/blood.2021012764","article-title":"An improved index for diagnosis and mortality prediction in malignancy-associated hemophagocytic lymphohistiocytosis","volume":"139","author":"Murakami","year":"2022","journal-title":"Blood"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"101515","DOI":"10.1016\/j.berh.2020.101515","article-title":"Hemophagocytic lymphohistiocytosis: An update on pathogenesis, diagnosis, and therapy","volume":"34","author":"Griffin","year":"2020","journal-title":"Best Pract. Res. Clin. Rheumatol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/j.ddmod.2019.10.008","article-title":"Chediak-Higashi syndrome: A review of the past, present, and future","volume":"31","author":"Sharma","year":"2020","journal-title":"Drug Discov. Today Dis. Model."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1080\/15513815.2022.2077489","article-title":"Chediak Higashi Syndrome with Hemophagocytic Lymphohistiocytosis","volume":"42","author":"Safavi","year":"2023","journal-title":"Fetal Pediatr. Pathol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"100091","DOI":"10.1016\/j.nerep.2022.100091","article-title":"Griscelli syndrome type 2: A well defined diagnosis with an intriguing diagnostic way","volume":"2","author":"Alessandra","year":"2022","journal-title":"Neuroimmunol. Rep."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"76","DOI":"10.5582\/irdr.2016.01084","article-title":"Griscelli syndrome subtype 2 with hemophagocytic lympho-histiocytosis: A case report and review of literature","volume":"6","author":"Minocha","year":"2017","journal-title":"Intractable Rare Dis. Res."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1093\/rheumatology\/key006","article-title":"Macrophage activation syndrome in adults: Recent advances in pathophysiology, diagnosis and treatment","volume":"58","author":"Carter","year":"2019","journal-title":"Rheumatology"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1503","DOI":"10.1016\/S0140-6736(13)61048-X","article-title":"Adult haemophagocytic syndrome","volume":"383","author":"Khamashta","year":"2014","journal-title":"Lancet"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"2989","DOI":"10.1007\/s00277-023-05271-w","article-title":"Delay in treatment of adult hemophagocytic lymphohistiocytosis is associated with worse in-hospital outcomes","volume":"102","author":"Abdelhay","year":"2023","journal-title":"Ann. Hematol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1002\/jha2.405","article-title":"Secondary hemophagocytic lymphohistiocytosis associated with Rocky Mountain spotted fever in a toddler: A case report","volume":"3","author":"Lim","year":"2022","journal-title":"EJHaem"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1258\/td.2010.100056","article-title":"Scrub typhus associated macrophage activation syndrome","volume":"40","author":"Gopal","year":"2010","journal-title":"Trop. Doct."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"e00813","DOI":"10.1016\/j.idcr.2020.e00813","article-title":"Ehrlichiosis Presenting as Hemophagocytic Lymphohistiocytosis in an Immunocompetent Adult","volume":"20","author":"Patel","year":"2020","journal-title":"IDCases"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"e99","DOI":"10.1590\/s1678-9946202062099","article-title":"Fatal murine typhus with hemophagocytic lymphohistiocytosis in a child","volume":"62","year":"2020","journal-title":"Rev. Inst. Med. Trop. Sao Paulo"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"e04744","DOI":"10.1002\/ccr3.4744","article-title":"The unusual case of babesiosis causing disseminated intravascular coagulation with hemophagocytic lymphohistiocytosis","volume":"9","author":"Goodman","year":"2021","journal-title":"Clin. Case Rep."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1016\/j.revmed.2022.05.011","article-title":"Murine typhus complicated by sHLH mimicking adult-onset Still\u2019s disease","volume":"43","author":"Jacquot","year":"2022","journal-title":"Rev. Med. Interne"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1111\/bjh.17453","article-title":"Anaplasmosis with associated haemophagocytic lymphohistiocytosis","volume":"194","author":"Rea","year":"2021","journal-title":"Br. J. Haematol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"893","DOI":"10.3201\/eid2405.171802","article-title":"Heartland virus and hemophagocytic lymphohistiocytosis in immunocompromised patient, Missouri, USA","volume":"24","author":"Carlson","year":"2018","journal-title":"Emerg. Infect. Dis."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1016\/j.bcmd.2015.06.009","article-title":"Ehrlichia-Induced Hemophagocytic Lymphohistiocytosis: A Case Series and Review of Literature","volume":"55","author":"Otrock","year":"2015","journal-title":"Blood Cells Mol. Dis."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1016\/j.jiph.2017.01.002","article-title":"Fatal human anaplasmosis associated with macrophage activation syndrome in Greece and the Public Health response","volume":"10","author":"Tsiodras","year":"2017","journal-title":"J. Infect. Public Health"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"115","DOI":"10.7883\/yoken.67.115","article-title":"A case report of scrub typhus-associated hemophagocytic syndrome and a review of literature","volume":"67","author":"Lin","year":"2014","journal-title":"Jpn. J. Infect. Dis."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"e28436","DOI":"10.1002\/pbc.28436","article-title":"Incidence and treatment of hemophagocytic lymphohistiocytosis in hospitalized children with Ehrlichia infection","volume":"67","author":"Cabler","year":"2020","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"103366","DOI":"10.1016\/j.amsu.2022.103366","article-title":"A rare case of delayed anaplasma phagocytophilum-induced pancytopenia: A diagnostic conundrum","volume":"75","author":"Song","year":"2022","journal-title":"Ann. Med. Surg."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"e187","DOI":"10.1016\/j.chest.2020.06.003","article-title":"A 58-Year-Old Man With Acute Encephalopathy, Fever, and Multi-Organ Dysfunction","volume":"158","author":"Rogers","year":"2020","journal-title":"Chest"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"782","DOI":"10.1111\/joim.13638","article-title":"Neoehrlichia mikurensis\u2014An emerging opportunistic tick-borne infection in immunosuppressed patients","volume":"293","author":"Gynthersen","year":"2023","journal-title":"J. Intern. Med."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"102747","DOI":"10.1016\/j.transci.2020.102747","article-title":"Q fever as a rare cause of hemophagocytic lymphohistiocytosis: Case report","volume":"59","author":"Albayrak","year":"2020","journal-title":"Transfus. Apher. Sci."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/j.patbio.2013.10.002","article-title":"Syndrome d\u2019activation macrophagique: Complication rare de typhus murin","volume":"62","author":"Loussaief","year":"2014","journal-title":"Pathol. Biol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1002\/ajh.2830500113","article-title":"Pancytopenia due to hemophagocytic syndrome as the presenting manifestation of babesiosis","volume":"50","author":"Gupta","year":"1995","journal-title":"Am. J. Hematol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"e253082","DOI":"10.1136\/bcr-2022-253082","article-title":"Secondary hemophagocytic lymphohistiocytosis due to Heartland virus","volume":"15","author":"Ahlers","year":"2022","journal-title":"BMJ Case Rep."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1016\/j.ijid.2017.09.026","article-title":"Babesia microti infection and hemophagocytic lymphohistiocytosis in an immunocompetent patient","volume":"65","author":"Go","year":"2017","journal-title":"Int. J. Infect. Dis."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"e20154176","DOI":"10.1542\/peds.2015-4176","article-title":"Use of extracorporeal support in hemophagocytic lymphohistiocytosis secondary to ehrlichiosis","volume":"138","author":"Cheng","year":"2016","journal-title":"Pediatrics"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"ofv125","DOI":"10.1093\/ofid\/ofv125","article-title":"Coxiella burnetii infection with severe hyperferritinemia in an asplenic patient","volume":"2","author":"Paine","year":"2015","journal-title":"Open Forum Infect. Dis."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1076","DOI":"10.1016\/j.arcped.2016.07.008","article-title":"Syndrome d\u2019activation macrophagique compliquant une fi\u00e8vre boutonneuse m\u00e9diterran\u00e9enne: Une observation p\u00e9diatrique","volume":"23","author":"Hsairi","year":"2016","journal-title":"Arch. Pediatr."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"2324709618758350","DOI":"10.1177\/2324709618758350","article-title":"Severe Human Granulocytic Anaplasmosis With Significantly Elevated Ferritin Levels in an Immunocompetent Host in Pennsylvania: A Case Report","volume":"6","author":"Camacci","year":"2018","journal-title":"J. Investig. Med. High Impact Case Rep."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1099\/jmm.0.025833-0","article-title":"Haemophagocytic syndrome and rickettsial diseases","volume":"60","author":"Cascio","year":"2011","journal-title":"J. Med. Microbiol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1097\/MPH.0000000000000400","article-title":"Hemophagocytic lymphohistiocytosis due to rickettsia japonica in a 3-month-old infant","volume":"37","author":"Otsuki","year":"2015","journal-title":"J. Pediatr. Hematol. Oncol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"961","DOI":"10.1016\/j.trstmh.2009.04.003","article-title":"Unusual pancytopenia secondary to haemophagocytosis syndrome in rickettsioses","volume":"103","author":"Premaratna","year":"2009","journal-title":"Trans. R. Soc. Trop. Med. Hyg."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"e6730","DOI":"10.1002\/ccr3.6730","article-title":"Hemophagocytic Lymphohistiocytosis secondary to Rickettsial infection: A case report","volume":"10","author":"Sharma","year":"2022","journal-title":"Clin. Case Rep."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"992","DOI":"10.3201\/eid2905.221488","article-title":"Fatal Case of Heartland Virus Disease Acquired in the Mid-Atlantic Region, United States","volume":"29","author":"Liu","year":"2023","journal-title":"Emerg. Infect. Dis."},{"key":"ref_44","first-page":"17947","article-title":"Two Infectious Agents Causing Hemophagocytic Lymphohistiocytosis","volume":"13","author":"Atiq","year":"2021","journal-title":"Cureus"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1007\/s12288-013-0299-3","article-title":"Macrophage activation syndrome secondary to human monocytic ehrlichiosis","volume":"30","author":"Kumar","year":"2014","journal-title":"Indian J. Hematol. Blood Transfus."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1002\/pbc.22814","article-title":"Ehrlichia-induced hemophagocytic lymphohistiocytosis in two children","volume":"56","author":"Hanson","year":"2011","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1029","DOI":"10.1093\/clinids\/21.4.1029","article-title":"Hemophagocytosis: A complication of acute Q fever in a child","volume":"21","author":"Hufnagel","year":"1995","journal-title":"Clin. Infect. Dis."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"420","DOI":"10.1016\/j.jaccas.2019.12.042","article-title":"Human Monocytic Ehrlichiosis Associated With Myocarditis and Hemophagocytic Lymphohistiocytosis","volume":"2","author":"Dahm","year":"2020","journal-title":"JACC Case Rep."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"918","DOI":"10.1093\/cid\/ciab667","article-title":"Donor-derived Ehrlichiosis: 2 Clusters Following Solid Organ Transplantation","volume":"74","author":"Saha","year":"2022","journal-title":"Clin. Infect. Dis."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1007\/s15010-021-01693-8","article-title":"A severe case of Israeli spotted fever with pleural effusion in Italy","volume":"50","author":"Guccione","year":"2022","journal-title":"Infection"},{"key":"ref_51","doi-asserted-by":"crossref","unstructured":"Akel, T., and Mobarakai, N. (2017). Hematologic manifestations of babesiosis. Ann. Clin. Microbiol. Antimicrob., 16.","DOI":"10.1186\/s12941-017-0179-z"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"E1685","DOI":"10.1503\/cmaj.220638","article-title":"Human granulocytic anaplasmosis complicated by hemophagocytic syndrome and coinfection","volume":"194","author":"Ladha","year":"2022","journal-title":"Can. Med. Assoc. J."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1016\/j.amjms.2020.08.029","article-title":"Hemophagocytic Lymphohistiocytosis Secondary to Ehrlichia Chaffeensis in Adults: A Case Series From Oklahoma","volume":"361","author":"Yuen","year":"2021","journal-title":"Am. J. Med. Sci."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"ofad213","DOI":"10.1093\/ofid\/ofad213","article-title":"Anaplasmosis-Induced Hemophagocytic Lymphohistiocytosis: A Case Report and Review of the Literature","volume":"10","author":"Scribner","year":"2023","journal-title":"Open Forum Infect. Dis."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1007\/s11684-013-0292-0","article-title":"Hemophagocytic lymphohistiocytosis: Critical reappraisal of a potentially under-recognized condition","volume":"7","author":"Padhi","year":"2013","journal-title":"Front. Med. China"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"1755","DOI":"10.1007\/s00277-017-3085-8","article-title":"Hemophagocytic lymphohistiocytosis (HLH) secondary to Ehrlichia chaffeensis with bone marrow involvement","volume":"96","author":"Naqash","year":"2017","journal-title":"Ann. Hematol."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1093\/tropej\/fms030","article-title":"Life-threatening scrub typhus with hemophagocytosis and acute respiratory distress syndrome in an infant","volume":"59","author":"Kwon","year":"2013","journal-title":"J. Trop. Pediatr."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1258\/td.2010.100303","article-title":"Rickettsial infection with hemophagocytosis","volume":"41","author":"Jayakrishnan","year":"2011","journal-title":"Trop. Doct."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"e13621","DOI":"10.1111\/tid.13621","article-title":"Ehrlichia-induced hemophagocytic lymphohistiocytosis after autologous stem cell transplant","volume":"23","author":"Mitma","year":"2021","journal-title":"Transpl. Infect. Dis."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"101468","DOI":"10.1016\/j.ttbdis.2020.101468","article-title":"Severe anaplasmosis represents a treatable cause of secondary hemophagocytic lymphohistiocytosis: Two cases and review of literature","volume":"11","author":"Rocco","year":"2020","journal-title":"Ticks Tick. Borne Dis."},{"key":"ref_61","first-page":"456","article-title":"A typical babesiosis in an immunocompetent patient","volume":"79","author":"Voisin","year":"2021","journal-title":"Ann. Biol. Clin."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"777","DOI":"10.4269\/ajtmh.16-0309","article-title":"Human granulocytic anaplasmosis as a cause of febrile illness in Korea since at least 2006","volume":"96","author":"Yi","year":"2017","journal-title":"Am. J. Trop. Med. Hyg."},{"key":"ref_63","first-page":"280","article-title":"Ehrlichiosis presenting with toxic shock-like syndrome and secondary hemophagocytic lymphohistiocytosis","volume":"109","author":"Pandey","year":"2013","journal-title":"J. Ark. Med. Soc."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/j.diagmicrobio.2016.08.007","article-title":"Human monocytic ehrlichiosis complicated by hemophagocytic lymphohistiocytosis and multi-organ dysfunction syndrome","volume":"86","author":"Kaplan","year":"2016","journal-title":"Diagn. Microbiol. Infect. Dis."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"1119","DOI":"10.1080\/00365540600684405","article-title":"Acute Q fever with hemophagocytic syndrome: Case report and literature review","volume":"38","author":"Chen","year":"2006","journal-title":"Scand. J. Infect. Dis."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1007\/s00277-017-3220-6","article-title":"Low-virulent Babesia venatorum infection masquerading as hemophagocytic syndrome","volume":"97","author":"Lazarevic","year":"2018","journal-title":"Ann. Hematol."},{"key":"ref_67","first-page":"003073","article-title":"Haemophagocytic Lymphohistiocytosis as a Complication of Israeli Spotted Fever","volume":"9","author":"Andrade","year":"2022","journal-title":"Eur. J. Case Rep. Intern. Med."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"301","DOI":"10.4103\/0022-3859.70949","article-title":"Hemophagocytosis in scrub typhus","volume":"56","author":"Valsalan","year":"2010","journal-title":"J. Postgrad. Med."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"ofaa355","DOI":"10.1093\/ofid\/ofaa355","article-title":"A Case of Hemophagocytic Lymphohistiocytosis Associated With Mediterranean Spotted Fever in a Healthy 29-Year-Old Female","volume":"7","author":"Karra","year":"2020","journal-title":"Open Forum Infect. Dis."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"e142","DOI":"10.1097\/MPH.0b013e3181c80ab9","article-title":"Hemophagocytic lymphohistiocytosis secondary to ehrlichia chaffeensis infection: A case report","volume":"32","author":"Burns","year":"2010","journal-title":"J. Pediatr. Hematol. Oncol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"1597","DOI":"10.2169\/internalmedicine.56.6904","article-title":"Hemophagocytic lymphohistiocytosis in a fatal case of severe fever with thrombocytopenia syndrome","volume":"56","author":"Nakano","year":"2017","journal-title":"Intern. Med."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1182\/blood.2019004149","article-title":"Hemophagocytic lymphohistiocytosis secondary to babesiosis","volume":"135","author":"Dermawan","year":"2020","journal-title":"Blood"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1080\/08998280.2022.2039046","article-title":"Anaplasmosis-induced hemophagocytic lymphohistiocytosis","volume":"35","author":"Lopez","year":"2022","journal-title":"Bayl. Univ. Med. Cent. Proc."},{"key":"ref_74","first-page":"5521274","article-title":"Ehrlichiosis-Associated Hemophagocytic Lymphohistiocytosis: A Case Series and Review of the Literature","volume":"2023","author":"Hammoud","year":"2023","journal-title":"Case Rep. Hematol."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1016\/j.jpeds.2014.09.053","article-title":"Hemophagocytic lymphohistiocytosis induced by monocytic ehrlichiosis","volume":"166","author":"Statler","year":"2015","journal-title":"J. Pediatr."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1007\/s15010-011-0142-4","article-title":"Coxiella burnetii causing haemophagocytic syndrome: A rare complication of an unusual pathogen","volume":"39","author":"Harris","year":"2011","journal-title":"Infection"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1002\/ajh.25364","article-title":"Hemophagocytic lymphohistiocytosis secondary to Babesia in an immunocompetent adult","volume":"94","author":"Obayomi","year":"2019","journal-title":"Am. J. Hematol."},{"key":"ref_78","first-page":"e26943","article-title":"An Unusual Case of Ehrlichiosis Manifesting With Hyponatremia, Acute Encephalopathy, and Hemophagocytic Lymphohistiocytosis","volume":"14","author":"Bolling","year":"2022","journal-title":"Cureus"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1177\/0009922814529183","article-title":"Hemophagocytic lymphohistiocytosis secondary to ehrlichiosis in a child","volume":"54","author":"Vijayan","year":"2015","journal-title":"Clin. Pediatr."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"76","DOI":"10.4103\/jgid.jgid_116_16","article-title":"Hemophagocytic lymphohistiocytosis associated with anaplasmosis","volume":"9","author":"Johnson","year":"2017","journal-title":"J. Glob. Infect. Dis."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"e14134","DOI":"10.1111\/petr.14134","article-title":"Ehrlichia-induced hemophagocytic lymphohistiocytosis in a pediatric kidney transplant recipient","volume":"26","author":"Singh","year":"2022","journal-title":"Pediatr. Transplant."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"132","DOI":"10.3390\/idr15010014","article-title":"Flea-Borne Typhus Causing Hemophagocytic Lymphohistiocytosis: An Autopsy Case","volume":"15","author":"Chandramohan","year":"2023","journal-title":"Infect. Dis. Rep."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"e01570","DOI":"10.1016\/j.idcr.2022.e01570","article-title":"Hemagophagocytic lymphohistiocytosis associated with recurrent Babesiosis with Lyme disease co-infection in an immunocompromised host on anti-CD20 monoclonal antibody therapy: A case report","volume":"29","author":"Zahra","year":"2022","journal-title":"IDCases"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1532\/IJH97.07012","article-title":"Nationwide survey of hemophagocytic lymphohistiocytosis in Japan","volume":"86","author":"Ishii","year":"2007","journal-title":"Int. J. Hematol."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1002\/pbc.22359","article-title":"Hemophagocytic lymphohistiocytosis in Texas: Observations on ethnicity and race","volume":"54","author":"Niece","year":"2010","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"119","DOI":"10.3389\/fimmu.2019.00119","article-title":"The Immunology of Macrophage Activation Syndrome","volume":"10","author":"Crayne","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"876415","DOI":"10.3389\/fimmu.2022.876415","article-title":"The Outcome of Induction Therapy for EBV-Related Hemophagocytic Lymphohistiocytosis: A Model for Risk Stratification","volume":"13","author":"Cui","year":"2022","journal-title":"Front. Immunol."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"2729","DOI":"10.1111\/apa.15973","article-title":"Virus-triggered secondary hemophagocytic lymphohistiocytosis","volume":"110","author":"Imashuku","year":"2021","journal-title":"Acta Paediatr."},{"key":"ref_89","doi-asserted-by":"crossref","unstructured":"Dumic, I., Jevtic, D., Veselinovic, M., Nordstrom, C.W., Jovanovic, M., Mogulla, V., Veselinovic, E.M., Hudson, A., Simeunovic, G., and Petcu, E. (2022). Human Granulocytic Anaplasmosis-A Systematic Review of Published Cases. Microorganisms, 10.","DOI":"10.3390\/microorganisms10071433"},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"69","DOI":"10.2147\/JBM.S46255","article-title":"Hemophagocytic lymphohistiocytosis: Review of etiologies and management","volume":"5","author":"George","year":"2014","journal-title":"J. Blood Med."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1086\/525852","article-title":"Persistent and relapsing babesiosis in immunocompromised patients","volume":"46","author":"Krause","year":"2008","journal-title":"Clin. Infect. Dis."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1016\/j.cll.2009.10.004","article-title":"Human Ehrlichiosis and Anaplasmosis","volume":"30","author":"Ismail","year":"2010","journal-title":"Clin. Lab. Med."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"834","DOI":"10.1056\/NEJMoa1203378","article-title":"A new phlebovirus associated with severe febrile illness in Missouri","volume":"367","author":"McMullan","year":"2012","journal-title":"N. Engl. J. Med."},{"key":"ref_94","first-page":"270","article-title":"Heartland Virus Disease\u2014United States, 2012\u20132013","volume":"63","author":"Pastula","year":"2014","journal-title":"Morb. Mortal. Wkly. Rep."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"205","DOI":"10.6061\/clinics\/2016(04)05","article-title":"Clinical presentation of hemophagocytic lymphohistiocytosis in adults is less typical than in children","volume":"71","author":"Zhang","year":"2016","journal-title":"Clinics"},{"key":"ref_96","doi-asserted-by":"crossref","unstructured":"Dumic, I., Madrid, C., Rueda Prada, L., Nordstrom, C.W., Taweesedt, P.T., and Ramanan, P. (2020). Splenic Complications of Babesia microti Infection in Humans: A Systematic Review. Can. J. Infect. Dis. Med. Microbiol., 2020.","DOI":"10.1155\/2020\/6934149"},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"776","DOI":"10.4103\/0366-6999.228234","article-title":"Central Nervous System Involvement in Hemophagocytic Lymphohistiocytosis in Adults: A Retrospective Analysis of 96 Patients in a Single Center","volume":"131","author":"Song","year":"2018","journal-title":"Chin. Med. J."},{"key":"ref_98","doi-asserted-by":"crossref","unstructured":"Sekeyov\u00e1, Z., Danchenko, M., Filip\u010d\u00edk, P., and Fournier, P.E. (2019). Rickettsial infections of the central nervous system. PLoS Negl. Trop. Dis., 13.","DOI":"10.1371\/journal.pntd.0007469"},{"key":"ref_99","doi-asserted-by":"crossref","unstructured":"Cosiquien, R.J.S., Stojiljkovic, N., Nordstrom, C.W., Amadi, E., Lutwick, L., and Dumic, I. (2023). Anaplasma phagocytophilum Encephalitis: A Case Report and Literature Review of Neurologic Manifestations of Anaplasmosis. Infect. Dis. Rep., 15.","DOI":"10.3390\/idr15040035"},{"key":"ref_100","first-page":"e26811","article-title":"Atypical Presentation of Babesiosis With Neurological Manifestations as Well as Hematological Manifestations","volume":"14","author":"Venigalla","year":"2022","journal-title":"Cureus"},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"373","DOI":"10.3109\/09273948.2013.848907","article-title":"Visual loss associated with rickettsial disease","volume":"22","author":"Kahloun","year":"2014","journal-title":"Ocul. Immunol. Inflamm."},{"key":"ref_102","first-page":"80","article-title":"Rickettsiosis associated with cerebral infarction: A new case study","volume":"26","author":"Boulahri","year":"2017","journal-title":"Pan Afr. Med. J."},{"key":"ref_103","doi-asserted-by":"crossref","unstructured":"Kularatne, S.A.M., Fernando, R., Selvaratnam, S., Narampanawa, C., Weerakoon, K., Wickramasinghe, S., Pathirage, M., Weerasinghe, V., Bandara, A., and Rajapakse, J. (2018). Intra-aural tick bite causing unilateral facial nerve palsy in 29 cases over 16 years in Kandy, Sri Lanka: Is rickettsial aetiology possible?. BMC Infect. Dis., 18.","DOI":"10.1186\/s12879-018-3338-8"},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"851","DOI":"10.1053\/j.ajkd.2014.10.012","article-title":"Acute kidney injury in adults with hemophagocytic lymphohistiocytosis","volume":"65","author":"Aulagnon","year":"2015","journal-title":"Am. J. Kidney Dis."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"e2025577","DOI":"10.1001\/jamanetworkopen.2020.25577","article-title":"Assessment of Risk Factors and Outcomes of Severe Ehrlichiosis Infection","volume":"3","author":"Kuriakose","year":"2020","journal-title":"JAMA Netw. Open"},{"key":"ref_106","first-page":"2323","article-title":"Tick-Borne Disease","volume":"71","author":"Bratton","year":"2005","journal-title":"Am. Fam. Physician"},{"key":"ref_107","first-page":"e27187","article-title":"Clustered Incidence of Leukocytoclastic Vasculitis and Purpura Fulminans: A Case Series of a Rare Dermatological Manifestation of Rickettsial Disease","volume":"14","author":"Pinto","year":"2022","journal-title":"Cureus"},{"key":"ref_108","first-page":"530","article-title":"Tickborne Diseases: Diagnosis and Management","volume":"101","author":"Pace","year":"2020","journal-title":"Am. Fam. Physician"},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"17","DOI":"10.5045\/br.2021.2020323","article-title":"Current status of the diagnosis and treatment of hemophagocytic lymphohistiocytosis in adults","volume":"56","author":"Kim","year":"2021","journal-title":"Blood Res."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1016\/j.jaci.2015.06.048","article-title":"Genetic predisposition to hemophagocytic lymphohistiocytosis: Report on 500 patients from the Italian registry","volume":"137","author":"Cetica","year":"2016","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"4041","DOI":"10.1182\/blood-2011-03-278127","article-title":"How I treat hemophagocytic lymphohistiocytosis","volume":"118","author":"Jordan","year":"2011","journal-title":"Blood"},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"e561","DOI":"10.1093\/cid\/ciac717","article-title":"Severe Mycobacterial Immune Reconstitution Inflammatory Syndrome (IRIS) in Advanced Human Immunodeficiency Virus (HIV) Has Features of Hemophagocytic Lymphohistiocytosis and Requires Prolonged Immune Suppression","volume":"76","author":"Rocco","year":"2023","journal-title":"Clin. Infect. Dis."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"2728","DOI":"10.1182\/blood-2017-06-788349","article-title":"Confirmed efficacy of etoposide and dexamethasone in HLH treatment: Long-term results of the cooperative HLH-2004 study","volume":"130","author":"Bergsten","year":"2017","journal-title":"Blood"},{"key":"ref_114","unstructured":"Zimmer, A.J., and Simonsen, K.A. (2023). Babesiosis, StatPearls Publishing."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"2692","DOI":"10.1182\/blood-2017-10-808543","article-title":"Etoposide for HLH: The limits of efficacy","volume":"130","author":"Ehl","year":"2017","journal-title":"Blood"},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"2908","DOI":"10.1182\/blood-2015-01-551622","article-title":"How I treat hemophagocytic lymphohistiocytosis in the adult patient","volume":"125","author":"Schram","year":"2015","journal-title":"Blood"},{"key":"ref_117","unstructured":"Konkol, S., and Rai, M. (2023). Lymphohistiocytosis, StatPearls Publishing."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"269","DOI":"10.3324\/haematol.2018.198655","article-title":"Treatment outcomes and prognostic factors in adult patients with secondary hemophagocytic lymphohistiocytosis not associated with malignancy","volume":"104","author":"Yoon","year":"2019","journal-title":"Haematologica"},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"59977","DOI":"10.18632\/oncotarget.19230","article-title":"A systematic review of malignancy-associated hemophagocytic lymphohistiocytosis that needs more attentions","volume":"8","author":"Wang","year":"2017","journal-title":"Oncotarget"},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"e217","DOI":"10.1016\/S2352-3026(21)00366-5","article-title":"Malignancy-associated haemophagocytic lymphohistiocytosis","volume":"9","author":"Setiadi","year":"2022","journal-title":"Lancet Haematol."}],"container-title":["Infectious Disease Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2036-7449\/16\/2\/12\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T14:02:10Z","timestamp":1760104930000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2036-7449\/16\/2\/12"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,2,21]]},"references-count":120,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2024,4]]}},"alternative-id":["idr16020012"],"URL":"https:\/\/doi.org\/10.3390\/idr16020012","relation":{},"ISSN":["2036-7449"],"issn-type":[{"value":"2036-7449","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,2,21]]}}}