Demographic study of leukaemia presenting within the first 3 months of life in the Northern Health Region of England.
OBJECTIVE
To determine the incidence and outcome of congenital leukaemia.


METHODS
Retrospective population based study of putative leukaemia arising during the first 3 months of life over an 18 year period within the Northern Health Region of England.


RESULTS
Nine infants with putative leukaemia were identified. Five had acute leukaemia and four had transient myeloproliferative disorder (TMD). Trisomy 21, either as Down's syndrome or perhaps restricted to proliferating marrow cells, was present in all four infants with TMD. The incidence of congenital acute leukaemia was 8.6/10(6) live births/year, but would be less than half this value if only patients presenting within 4 weeks of birth were counted. Remission was induced in three of the five patients with acute leukaemia. One patient, who presented at birth, remains well five years after diagnosis. All four patients with TMD survive.


CONCLUSIONS
Congenital leukaemia is very rare but is not inevitably fatal. Finding trisomy 21 in spontaneously dividing blood or bone marrow cells of an infant with putative acute leukaemia, particularly within 3 months of birth, should encourage a cautious clinical approach and suggests that the diagnosis might be TMD.
Bajwa
R P S
RP
Department of Paediatric Oncology, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, UK.
Skinner
R
R
Windebank
K P
KP
Reid
M M
MM
D000208:Acute Disease; D004314:Down Syndrome / Q000150:complications; D004739:England / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007938:Leukemia / Q000151:congenital* / Q000453:epidemiology*; D008297:Male; D009196:Myeloproliferative Disorders / Q000150:complications / Q000453:epidemiology; D011379:Prognosis; D012189:Retrospective Studies