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Hyperleucocytosis in paediatric acute myeloid leukaemia - the challenge of white blood cell counts above 200 × 10(9) /l. The NOPHO experience 1984-2014.

Artikel i vetenskaplig tidskrift
Författare Bernward Zeller
Heidi Glosli
Erik Forestier
Shau-Yin Ha
Kirsi Jahnukainen
Ólafur G Jónsson
Birgitte Lausen
Josefine Palle
Henrik Hasle
Jonas Abrahamsson
Publicerad i British journal of haematology
Volym 178
Nummer/häfte 3
Sidor 448–456
ISSN 1365-2141
Publiceringsår 2017
Publicerad vid Institutionen för kliniska vetenskaper, sektionen för kvinnors och barns hälsa, Avdelningen för pediatrik
Sidor 448–456
Språk en
Länkar dx.doi.org/10.1111/bjh.14692
www.ncbi.nlm.nih.gov/entrez/query.f...
Ämneskategorier Pediatrik

Sammanfattning

Hyperleucocytosis in paediatric acute myeloid leukaemia (AML) is associated with increased morbidity and mortality. We studied hyperleucocytosis in 890 patients with AML aged 0-18 years registered in the Nordic Society of Paediatric Haematology and Oncology (NOPHO) registry, with special focus on very high white blood cell counts (WBC >200 × 10/l). Eighty-six patients (10%) had WBC 100-199 × 10(9) /l and 57 (6%) had WBC ≥200 × 10(9) /l. Patients with WBC ≥200 × 10(9) /l had a high frequency of t(9;11) and a paucity of trisomy 8. Due to the high frequency of deaths within the first 2 weeks (30% vs. 1% for all others), overall survival in this group was inferior to patients with WBC <200 × 10(9) /l (39% vs. 61%). Main cause of early death was intracranial haemorrhage and leucostasis. Twenty-six per cent of these patients never started antileukaemic protocol therapy. Leukapheresis or exchange transfusion was used in 24% of patients with hyperleucocytosis without impact on survival. Patients with hyperleucocytosis surviving the first week had identical survival as patients with lower WBC. We conclude that death within the first days after diagnosis is the major challenge in patients with high WBC and advocate rapid initiation of intensive chemotherapy.

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