{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T21:14:14Z","timestamp":1790802854207,"version":"4.1.0"},"reference-count":56,"publisher":"Georg Thieme Verlag KG","issue":"06","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Klin Padiatr"],"published-print":{"date-parts":[[2021,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Children with Down syndrome are at a high risk of developing transient abnormal myelopoiesis (TAM; synonym: TMD) or myeloid leukemia (ML-DS). While most patients with TAM are asymptomatic and go into spontaneous remission without a need for therapy, around 20% of patients die within the first six months due to TAM-related complications. Another 20\u201330% of patients progress from TAM to ML-DS. ML-DS patients are particularly vulnerable to therapy-associated toxicity, but the prognosis of relapsed ML-DS is extremely poor \u2013 thus, ML-DS therapy schemata must strive for a balance between appropriate efficacy (to avoid relapses) and treatment-related toxicity. This guideline presents diagnostic and therapeutic strategies for TAM and ML-DS based on the experience and results of previous clinical studies from the BFM working group, which have helped reduce the risk of early death in symptomatic TAM patients using low-dose cytarabine, and which have achieved excellent cure rates for ML-DS using intensity-reduced treatment protocols.<\/jats:p>","DOI":"10.1055\/a-1532-2016","type":"journal-article","created":{"date-parts":[[2021,8,18]],"date-time":"2021-08-18T23:29:57Z","timestamp":1629329397000},"page":"267-277","source":"Crossref","is-referenced-by-count":14,"title":["Recommendations for Diagnosis and Treatment of Children with Transient Abnormal Myelopoiesis (TAM) and Myeloid Leukemia in Down Syndrome (ML-DS)"],"prefix":"10.1055","volume":"233","author":[{"given":"Sina","family":"Al-Kershi","sequence":"additional","affiliation":[{"name":"Clinic for Pediatrics, University Hospital Frankfurt, Frankfurt, Germany"},{"name":"Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8582-5006","authenticated-orcid":false,"given":"Richard","family":"Golnik","sequence":"additional","affiliation":[{"name":"Clinic for Pediatrics, University Hospital Frankfurt, Frankfurt, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marius","family":"Flasinski","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, Psychosomatic Medicine and Psychotherapy, Hospital Tauberbischofsheim, Tauberbischofsheim, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katharina","family":"Waack","sequence":"additional","affiliation":[{"name":"Pediatrics III, Pediatric Hematology and Oncology, University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mareike","family":"Rasche","sequence":"additional","affiliation":[{"name":"Pediatrics III, Pediatric Hematology and Oncology, University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ursula","family":"Creutzig","sequence":"additional","affiliation":[{"name":"Pediatric Hematology and Oncology, Hannover Medical School, Hannover, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Dworzak","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, St. Anna Children\u2019s Hospital and Children\u2019s Cancer Research Institute, Wien, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dirk","family":"Reinhardt","sequence":"additional","affiliation":[{"name":"Pediatrics III, Pediatric Hematology and Oncology, University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1070-0727","authenticated-orcid":false,"given":"Jan-Henning","family":"Klusmann","sequence":"additional","affiliation":[{"name":"Clinic for Pediatrics, University Hospital Frankfurt, Frankfurt, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2021,8,18]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1007\/s00277-005-0045-5","article-title":"Optimal treatment intensity in children with Down syndrome and myeloid leukaemia: data from 56 children treated on NOPHO-AML protocols and a review of the literature","volume":"85","author":"L Abildgaard","year":"2006","journal-title":"Ann Hematol"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"2480","DOI":"10.1182\/blood-2003-10-3383","article-title":"Natural history of GATA1 mutations in Down syndrome","volume":"103","author":"M 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