{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,28]],"date-time":"2025-08-28T00:06:34Z","timestamp":1756339594069,"version":"3.44.0"},"reference-count":9,"publisher":"BMJ","issue":"11","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2023,11,9]]},"published-print":{"date-parts":[[2023,11]]},"abstract":"<jats:p>Paediatric primary myelofibrosis (PMF) is exceedingly rare and distinct compared with adult PMF. It is characterised by peripheral blood cytopenias, leucoerythroblastosis, reticulin fibrosis, extramedullary haematopoiesis and hepatosplenomegaly. In the absence of laid down diagnostic criteria, the diagnosis is largely of exclusion. Though early haematological stem cell transplant (HSCT) remains the treatment of choice, spontaneous remission or remission with steroids and\/or cytoreductive agents is described in around 20% of cases of paediatric PMF. Moreover, HSCT in paediatric PMF is associated with high mortality (30%\u201345%). Therefore, it may be prudent to consider a trial of steroids and\/or cytoreductive agents in all transfusion-dependent paediatric PMF while considering HSCT and ongoing bone marrow donor search. We describe one such infant with PMF who had complete remission of clinical and haematological parameters with a combination therapy of steroids and hydroxyurea.<\/jats:p>","DOI":"10.1136\/bcr-2023-256210","type":"journal-article","created":{"date-parts":[[2023,11,22]],"date-time":"2023-11-22T12:30:34Z","timestamp":1700656234000},"page":"e256210","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Complete resolution of primary myelofibrosis in an infant with steroids and hydroxyurea"],"prefix":"10.1136","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4754-8340","authenticated-orcid":false,"given":"Sanjeev","family":"Khera","sequence":"first","affiliation":[{"name":"Pediatrics, Army Hospital Research and Referral, New Delhi, Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Priyanka","family":"Misra","sequence":"additional","affiliation":[{"name":"Pathology, Command Hospital Kolkata, Kolkata, West Bengal, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kanwaljeet","family":"Singh","sequence":"additional","affiliation":[{"name":"Pathology, Command Hospital Kolkata, Kolkata, West Bengal, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Preeti","family":"Tripathi","sequence":"additional","affiliation":[{"name":"Pathology, Army Hospital Research and Referral, New Delhi, Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2023,11,22]]},"reference":[{"key":"2025082712024321000_16.11.e256210.1","doi-asserted-by":"publisher","DOI":"10.1007\/s12308-015-0256-1"},{"key":"2025082712024321000_16.11.e256210.2","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2012.12"},{"key":"2025082712024321000_16.11.e256210.3","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23140"},{"key":"2025082712024321000_16.11.e256210.4","doi-asserted-by":"crossref","first-page":"e120","DOI":"10.1097\/MPH.0b013e31828800cc","article-title":"Stem cell transplantation in primary myelofibrosis of childhood","volume":"35","author":"Mitton","year":"2013","journal-title":"J Pediatr Hematol Oncol"},{"key":"2025082712024321000_16.11.e256210.5","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1097\/MPH.0b013e31829cdea0","article-title":"Idiopathic myelofibrosis in children: primary myelofibrosis, essential Thrombocythemia, or transient process","volume":"35","author":"Slone","year":"2013","journal-title":"J Pediatr Hematol Oncol"},{"key":"2025082712024321000_16.11.e256210.6","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-07-575373"},{"key":"2025082712024321000_16.11.e256210.7","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-07-170449"},{"key":"2025082712024321000_16.11.e256210.8","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1016\/j.bjhh.2016.04.003","article-title":"Primary myelofibrosis: Current therapeutic options","volume":"38","author":"de Melo Campos","year":"2016","journal-title":"Rev Bras Hematol Hemoter"},{"key":"2025082712024321000_16.11.e256210.9","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-199805000-00016"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2023-256210","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T19:02:48Z","timestamp":1756321368000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2023-256210"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11]]},"references-count":9,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2023,11,22]]},"published-print":{"date-parts":[[2023,11]]}},"alternative-id":["10.1136\/bcr-2023-256210"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2023-256210","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2023,11]]}}}