{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T23:51:42Z","timestamp":1788652302760,"version":"build-2803163510"},"reference-count":45,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2010,8,30]],"date-time":"2010-08-30T00:00:00Z","timestamp":1283126400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hematological Oncology"],"published-print":{"date-parts":[[2011,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Hemophagocytic lymphohistiocytosis (HLH) is rare in adults and is usually fatal without treatment. We present a consecutive series of 18 adults with HLH diagnosed at our institution between 2004 and 2009. All diagnoses were confirmed by pathology. The median age at diagnosis was 56 years (range: 18\u201373 years), with a male: female ratio of 2:1. Patients uniformly presented with fever. Fifty\u2010five per cent of the patients presented with evidence of hepatomegaly or splenomegaly. All of the patients had at least a bi\u2010 or trilineage cytopenia. Elevated liver enzymes, hyperferritinemia, hypertriglyceridemia and hyperfibrinogenemia were seen in 50, 100, 40 and 50% of patients, respectively. The presumed causes were as follows; haematological malignancies (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20094), post\u2010autologous stem cell transplant (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20092), infection (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20092), rheumatologic illness (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20092), sickle cell disease (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20091), post\u2010orthotopic liver transplant (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20091) and idiopathic (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20093). The median time from suspicion to diagnosis was 5 days (1\u201327 days). Corticosteroids and\/or cyclosporine were the most frequently used treatment regimen. Other agents used were etoposide, IVIG, cyclophosphamide and chemotherapy. The mortality rate was 72%, with multi\u2010system organ failure being the most common cause of death. Median survival time from diagnosis was 35 days. Six patients are alive to date. In a univariate analysis, the presence of fever was the only factor that was statistically significant for predicting a poor prognosis (early mortality) (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.05). In conclusion, a high index of suspicion is the critical factor for early diagnosis. Early treatment with immunosuppressant is warranted, and a thorough diagnostic evaluation to identify the underlying cause should be undertaken. Copyright \u00a9 2010 John Wiley &amp; Sons, Ltd.\n                  <\/jats:p>","DOI":"10.1002\/hon.960","type":"journal-article","created":{"date-parts":[[2010,8,31]],"date-time":"2010-08-31T14:43:04Z","timestamp":1283265784000},"page":"100-106","source":"Crossref","is-referenced-by-count":93,"title":["Secondary hemophagocytic syndrome in adults: a case series of 18 patients in a single institution and a review of literature"],"prefix":"10.1002","volume":"29","author":[{"given":"Munira","family":"Shabbir","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Lucas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Lazarchick","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keisuke","family":"Shirai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2010,8,30]]},"reference":[{"key":"e_1_2_5_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2007.05.001"},{"issue":"10","key":"e_1_2_5_3_2","first-page":"633","article-title":"The hemophagocytic syndrome\/macrophage activation syndrome: a final common pathway of a cytokine storm","volume":"11","author":"Szyper\u2010Kravitz M","year":"2009","journal-title":"Isr Med Assoc J"},{"key":"e_1_2_5_4_2","doi-asserted-by":"publisher","DOI":"10.1182\/asheducation-2009.1.127"},{"key":"e_1_2_5_5_2","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-199302000-00012"},{"key":"e_1_2_5_6_2","doi-asserted-by":"publisher","DOI":"10.1136\/adc.43.231.601"},{"issue":"5","key":"e_1_2_5_7_2","first-page":"499","article-title":"Familial hemophagocytic lymphohistiocytosis: how late can the onset be?","volume":"86","author":"Allen M","year":"2001","journal-title":"Haematologica"},{"key":"e_1_2_5_8_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1991.tb11878.x"},{"issue":"2","key":"e_1_2_5_9_2","first-page":"197","article-title":"Hemophagocytic lymphohistiocytosis. 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