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Risk factors for early death in neonates with Down syndrome and transient leukaemia.

Abstract：

:Transient leukaemia (TL) in neonates with Down syndrome (DS) is characterized by the transient appearance of blast cells in the peripheral blood that resolves spontaneously. Some TL patients die at an early age due to organ failure. Seventy DS patients with TL were studied retrospectively to identify clinical and laboratory characteristics associated with early death (<6 months of age). Sixteen of 70 patients (22.9%) died early. The main causes of death were organ failure, particularly hepatic and cardiopulmonary failure. On univariate analysis, early gestational age (EGA), high white blood cell (WBC) count (> or =100 x 10(9)/l), percentage of peripheral blasts, elevated aspartate transaminase (AST), elevated direct bilirubin (DB), and low Apgar score were significantly associated with poor survival. On multivariate analysis, EGA, WBC count, and DB were independent predictors of poor outcome. A simple risk stratification system combining EGA and WBC count was devised to predict poor outcome. Term infants (EGA > or = 37 weeks) whose WBC count was lower than 100 x 10(9)/l had the best outcome [7.7% (3/39) died early], while preterm infants (EGA < 37 weeks) whose WBC count was higher than 100 x 10(9)/l had the worst outcome [54.5% (6/11) died early]. This stratification system may be useful for identifying high-risk patients who need early therapeutic interventions.

journal_name

Br J Haematol

journal_title

British journal of haematology

authors

Muramatsu H,Kato K,Watanabe N,Matsumoto K,Nakamura T,Horikoshi Y,Mimaya J,Suzuki C,Hayakawa M,Kojima S

doi

10.1111/j.1365-2141.2008.07231.x

subject

Has Abstract

pub_date

2008-08-01 00:00:00

pages

610-5

issue

4

eissn

0007-1048

issn

1365-2141

pii

BJH7231

journal_volume

142

pub_type

杂志文章

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