Przejściowy zespół mieloproliferacyjny – praca poglądowa

*Bartosz Chyżyński, Michał Matysiak

Summary
Children with Down syndrome (DS) are at increased risk of hematological disorders, in particular hematopoietic neoplastic diseases. A disorder characteristic for patients with DS is transient myeloproliferative disorder (TMD) occurring in about 30% of newborns with DS. The development of TMD occurs as a result of the cooperation of two genetic disorders – trisomy 21 (T21) and mutation of the GATA1 transcription factor gene. The clinical course of TMD varies from clinically silent to symptomatic with the occurrence of life-threatening complications. Most patients have spontaneous remission within 3 months without treatment. Patients who develop life-threatening complications may require cytostatic treatment. About 10-20% of patients who develop TMD during the neonatal period in the first 4 years of life will develop acute myeloid leukemia in Down syndrome (myeloid leukemia of Down syndrome – ML-DS). Due to the high risk of developing ML-DS, patients who have been diagnosed with TMD during the neonatal period should be under close hematological care up to 4 years of age.

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