{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T21:03:45Z","timestamp":1781816625026,"version":"3.54.5"},"reference-count":11,"publisher":"BMJ","issue":"5","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2020,4,7]]},"published-print":{"date-parts":[[2020,5]]},"abstract":"<jats:p>Haemophagocytic lymphohistiocytosis (HLH) can be a rapidly fatal disease. Current treatment in adults is extrapolated from the HLH-2004 protocol that specifies a regimen of etoposide, dexamethasone and cyclosporine. However, HLH presents as a spectrum of disease severity. A therapeutic challenge arises for milder cases where the harms of potent chemotherapy such as etoposide may outweigh its benefit. We present a case of an adult with HLH who developed significant pancytopenia but was otherwise not critically ill and who responded to treatment with a chemotherapy-sparing approach consisting of intravenous immunoglobulins and corticosteroids alone. The case illustrates that tailored therapy may allow effective treatment of the disorder while minimising therapy-related toxicities.<\/jats:p>","DOI":"10.1136\/bcr-2020-234490","type":"journal-article","created":{"date-parts":[[2020,5,6]],"date-time":"2020-05-06T06:14:25Z","timestamp":1588745665000},"page":"e234490","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":2,"title":["Chemotherapy-sparing treatment of haemophagocytic lymphohistiocytosis with intravenous immunoglobulins and corticosteroids"],"prefix":"10.1136","volume":"13","clinical-trial-number":[{"clinical-trial-number":"nct02400463","registry":"10.18810\/clinical-trials-gov"}],"author":[{"given":"Evan C","family":"Chen","sequence":"first","affiliation":[{"name":"Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA"},{"name":"Division of Hematology\/Oncology, Massachusetts General Hospital, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan A","family":"Stefely","sequence":"additional","affiliation":[{"name":"Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bimalangshu R","family":"Dey","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Massachusetts General Hospital, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Walter H","family":"Dzik","sequence":"additional","affiliation":[{"name":"Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA"},{"name":"Hematology and Blood Transfusion Service, Massachusetts General Hospital, Boston, MA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2020,5,5]]},"reference":[{"key":"2025082703035334000_13.5.e234490.1","doi-asserted-by":"publisher","DOI":"10.1126\/science.286.5446.1957"},{"key":"2025082703035334000_13.5.e234490.2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-02-074468"},{"key":"2025082703035334000_13.5.e234490.3","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21039"},{"key":"2025082703035334000_13.5.e234490.4","doi-asserted-by":"crossref","first-page":"2613","DOI":"10.1002\/art.38690","article-title":"Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome","volume":"66","author":"Fardet","year":"2014","journal-title":"Arthritis Rheumatol"},{"key":"2025082703035334000_13.5.e234490.5","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/aqw076"},{"key":"2025082703035334000_13.5.e234490.6","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmoldx.2016.02.003"},{"key":"2025082703035334000_13.5.e234490.7","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2017-06-788349"},{"key":"2025082703035334000_13.5.e234490.8","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.1141"},{"key":"2025082703035334000_13.5.e234490.9","unstructured":"Dunogu\u00e9 B , Gerin M , Cl L , et al . Intravenous immunoglobulin therapy for secondary hemophagocytic lymphohistiocytosis: a retrospective study of 46 patients. ACR\/ARHP Annual Meeting 2014;2199."},{"key":"2025082703035334000_13.5.e234490.10","unstructured":"American Society of Health-System Pharmacists . Current drug shortages: immune globulin, intravenous or subcutaneous (human), 2020. Available: https:\/\/www.ashp.org\/drug-shortages\/current-shortages\/Drug-Shortage-Detail.aspx?id=527 [Accessed 28 Mar 2020]."},{"key":"2025082703035334000_13.5.e234490.11","doi-asserted-by":"crossref","first-page":"e630","DOI":"10.1016\/S2352-3026(19)30156-5","article-title":"Ruxolitinib in adult patients with secondary haemophagocytic lymphohistiocytosis: an open-label, single-centre, pilot trial","volume":"6","author":"Ahmed","year":"2019","journal-title":"Lancet Haematol"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2020-234490","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T10:04:03Z","timestamp":1756289043000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2020-234490"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,5]]},"references-count":11,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2020,5,5]]},"published-print":{"date-parts":[[2020,5]]}},"alternative-id":["10.1136\/bcr-2020-234490"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2020-234490","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,5]]}}}