Down sendromlu bir yenidoğanda geçici myeloproliferatif hastalık
Küçük Resim Yok
Tarih
2013
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info:eu-repo/semantics/openAccess
Özet
Geçici myeloproliferatif hastalık (GMPH), Trizomi 21 (Down Sendromu) tanısı ile izlenen infantların yaklaşık %10’unda ortaya çıkabilen, periferik kanda myeloid blastların artımı ile karakterize bir durumdur. GMPH, Down Sendromun’nun işaretlerini taşımayan mozaik trizomi 21 hastalarında da görülebilir. GMPH’lı infantlarda ilk 3 yılda akut m ega¬karyoblastik lösemi (AML -M7) gelişme riski yaklaşık % 30’dur. Biz burada GMPH gelişen Down Sendromlu bir vaka sunduk. Down sendromlu yenidoğanlarda hepasopslenom egali, lökositoz veya trombositopeni ve periferik kan yayma incelemeside myeloblast saptanması durumunda GMPH düşünülmesinin önemine vurgu yapmak istedik .
Transient myeloproliferative disease (TMD), defined by circulating nonlymphoid blast in the peripheral blood, occurs in approximately 10% of infants with constitutional trisomy 21 (Down syndrome). The TMD phenotype may also occur in n ewborns who do not have clinical signs of Down syndrome but nonconstitutional trisomy 21 due to mosaicism. Acute mega-karyoblastic leukemia (AML -M7) develops in approx ima¬tely 30 % of the cases with TMD within the first three years of life. In this case report, a newborn with TMD with Down syndrome is presented. We emphasize the importa nce of the peripheral blood smear examination in newborns with DS, especially with hepatosplenomegaly, leukocytosis or throm¬bocytopenia and myeloblast of the peripheral smear, in order to diagnose TMD.
Transient myeloproliferative disease (TMD), defined by circulating nonlymphoid blast in the peripheral blood, occurs in approximately 10% of infants with constitutional trisomy 21 (Down syndrome). The TMD phenotype may also occur in n ewborns who do not have clinical signs of Down syndrome but nonconstitutional trisomy 21 due to mosaicism. Acute mega-karyoblastic leukemia (AML -M7) develops in approx ima¬tely 30 % of the cases with TMD within the first three years of life. In this case report, a newborn with TMD with Down syndrome is presented. We emphasize the importa nce of the peripheral blood smear examination in newborns with DS, especially with hepatosplenomegaly, leukocytosis or throm¬bocytopenia and myeloblast of the peripheral smear, in order to diagnose TMD.
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