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It can affect all age groups. Diagnostic evaluation of the patient with suspected\n                    <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>\n                    has to address three main questions: (i) does the patient have\n                    <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>\n                    ? There is no simple diagnostic test, but a number of clinical and laboratory criteria define this clinical syndrome. (ii) Can a trigger be identified? A variety of infections, malignant or autoimmune diseases can contribute to the disturbed immune homeostasis with important consequences for treatment. (iii) Does the patient suffer from a genetic disease predisposing to\n                    <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>\n                    ? Recent advances in the understanding of the genetic and pathophysiological basis of\n                    <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>\n                    have enabled a better and more rapid answer to this question, which is relevant for prognosis and the decision to perform haematopoietic stem cell transplantation. This review summarizes the current diagnostic approach to the patient with\n                    <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>\n                    .\n                  <\/jats:p>","DOI":"10.1111\/bjh.12138","type":"journal-article","created":{"date-parts":[[2012,12,4]],"date-time":"2012-12-04T15:04:55Z","timestamp":1354633495000},"page":"275-287","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":114,"title":["Diagnostic evaluation of patients with suspected haemophagocytic lymphohistiocytosis"],"prefix":"10.1111","volume":"160","author":[{"given":"Kai","family":"Lehmberg","sequence":"first","affiliation":[{"name":"Department of Paediatric Haematology and Oncology University Medical Centre Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephan","family":"Ehl","sequence":"additional","affiliation":[{"name":"Centre of Chronic Immunodeficiency University Hospital Freiburg Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2012,12,4]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21423"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2009.07885.x"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-1759(03)00265-5"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.22037"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.187.11.1903"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.22416"},{"key":"e_1_2_4_8_1","doi-asserted-by":"publisher","DOI":"10.1186\/ar3843"},{"key":"e_1_2_4_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-06-284935"},{"key":"e_1_2_4_10_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-02-074468"},{"key":"e_1_2_4_11_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-08-374199"},{"key":"e_1_2_4_12_1","first-page":"207","article-title":"Malignancy\u2010associated hemophagocytic lymphohistiocytosis in pediatric cases: a multicenter study from Turkey","volume":"51","author":"Celkan T.","year":"2009","journal-title":"The Turkish Journal of Pediatrics"},{"key":"e_1_2_4_13_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-04-1477"},{"key":"e_1_2_4_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/2424"},{"key":"e_1_2_4_15_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI40732"},{"key":"e_1_2_4_16_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e31824f800a"},{"key":"e_1_2_4_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(99)70423-3"},{"key":"e_1_2_4_18_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-11-4413"},{"key":"e_1_2_4_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.23415"},{"key":"e_1_2_4_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(03)00855-9"},{"key":"e_1_2_4_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.iac.2008.01.010"},{"key":"e_1_2_4_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.coi.2007.04.006"},{"key":"e_1_2_4_23_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.106.4.e58"},{"key":"e_1_2_4_24_1","doi-asserted-by":"publisher","DOI":"10.3109\/08880018.2011.601402"},{"key":"e_1_2_4_25_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.bor.0000381996.69261.71"},{"key":"e_1_2_4_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21564"},{"key":"e_1_2_4_27_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073808319073"},{"key":"e_1_2_4_28_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V78.11.2918.2918"},{"key":"e_1_2_4_29_1","first-page":"29","article-title":"Diagnostic guidelines for hemophagocytic lymphohistiocytosis. 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