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Exchange transfusion in patients with Down syndrome and severe transient leukemia
Author(s) -
Hayasaka Itaru,
Cho Kazutoshi,
Morioka Keita,
Kaneshi Yosuke,
Akimoto Takuma,
Furuse Yuta,
Moriichi Akinori,
Iguchi Akihiro,
Cho Yuko,
Minakami Hisanori,
Ariga Tadashi
Publication year - 2015
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.12586
Subject(s) - medicine , exchange transfusion , pediatrics , gestational age , white blood cell , tumor lysis syndrome , respiratory failure , hydrops fetalis , leukemia , surgery , gestation , pregnancy , chemotherapy , genetics , biology
Background Among neonates with Down syndrome ( DS ) and transient leukemia ( TL ), hyperleukocytosis (white blood cell [ WBC ] count >100 × 10 9 /L) is associated with increased blood viscosity, respiratory failure due to pulmonary hypertension, multiorgan failure, and increased risk of early death. There have been no previous studies focusing on the effects of exchange transfusion ( ExT ) on WBC count, respiratory status, and other parameters in TL patients with hyperleukocytosis. Methods An observational retrospective study was carried out at a single center of all five DS neonates with TL , GATA1 mutations, and hyperleukocytosis, born at a median gestational age of 34 weeks (range, 30–38 weeks) with birthweight 2556 g (range, 1756–3268 g) during a 24 month study period between S eptember 2011 and A ugust 2013. All five neonates underwent ExT at a median age of 2 days (range, 0–5 days) before initiation of other cytoreductive therapy with cytarabine, which was carried out in two patients. Results All patients required respiratory support before ExT . After ExT , respiration status improved in all five patients: WBC count (mean) decreased by 85% from 143 × 10 9 /L to 21 × 10 9 /L. None developed tumor lysis syndrome. Three survived and two died: one hydrops fetalis neonate born at gestational week 30 died at age 5 days, and another died eventually from acute gastroenteritis 40 days after leaving hospital at the age of 155 days with complete remission. Two of the three surviving neonates developed acute megakaryocytic leukemia at age 90 days and 222 days. Conclusion ExT was very effective in improving hyperleukocytosis and may have had favorable effects on respiration.