Recommendations for Diagnosis and Treatment of Children with Transient Abnormal Myelopoiesis (TAM) and Myeloid Leukemia in Down Syndrome (ML-DS).
Klin Padiatr
; 233(6): 267-277, 2021 Nov.
Article
em En
| MEDLINE
| ID: mdl-34407551
ABSTRACT
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 20-30% 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 - 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.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Leucemia Mieloide
/
Síndrome de Down
/
Reação Leucemoide
Tipo de estudo:
Diagnostic_studies
/
Guideline
/
Prognostic_studies
Limite:
Child
/
Humans
Idioma:
En
Revista:
Klin Padiatr
Ano de publicação:
2021
Tipo de documento:
Article
País de afiliação:
Alemanha