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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
Author(s) -
Zeller Bernward,
Glosli Heidi,
Forestier Erik,
Ha ShauYin,
Jahnukainen Kirsi,
Jónsson Ólafur G.,
Lausen Birgitte,
Palle Josefine,
Hasle Henrik,
Abrahamsson Jonas
Publication year - 2017
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.14692
Subject(s) - medicine , leukapheresis , white blood cell , hematology , myeloid leukaemia , chemotherapy , myeloid , pediatrics , gastroenterology , stem cell , genetics , cd34 , biology
Summary 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.