Congenital leukaemia: the Dutch experience and review of the literature

We reviewed Dutch patients and those described in the literature with congenital leukaemia in the past 25 years, with the intention to obtain an overview of the characteristics of this rare disease. Among the 117 patients reviewed, acute myeloid leukaemia (AML) was more frequent (64%) than acute lym...

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Bibliographic Details
Published in:British journal of haematology 2002-06, Vol.117 (3), p.513-524
Main Authors: Bresters, Dorine, Reus, Angelino C. W., Veerman, Anjo J. P., Van Wering, Elizabeth R., Van Der Does‐van den Berg, Anna, Kaspers, Gertjan J. L.
Format: Article
Language:English
Subjects:
Acute Disease
acute lymphoblastic leukaemia
acute myeloid leukaemia
Biological and medical sciences
Chromosome Aberrations
congenital leukaemia
Diagnosis, Differential
Female
Hematologic and hematopoietic diseases
Hematology
Humans
Infant, Newborn
Leukemia - congenital
Leukemia - diagnosis
Leukemia, Myeloid - congenital
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Male
Medical sciences
Myeloproliferative Disorders - diagnosis
Precursor Cell Lymphoblastic Leukemia-Lymphoma - congenital
Prognosis
transient myeloproliferative disorder
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Summary:We reviewed Dutch patients and those described in the literature with congenital leukaemia in the past 25 years, with the intention to obtain an overview of the characteristics of this rare disease. Among the 117 patients reviewed, acute myeloid leukaemia (AML) was more frequent (64%) than acute lymphoblastic leukaemia (ALL, 21%). Most patients had a high leukaemic cell load with hepatosplenomegaly, leukaemia cutis and hyperleucocytosis. Cytogenetic abnormalities were found in the majority of the patients tested (72%); 11q23 abnormalities were found in less than half of them (42%). The probability of overall survival at 24 months was only 23%. When congenital AML and ALL were compared, clinical characteristics and overall survival were not significantly different. However, in patients at risk, the probability of event‐free survival (EFS) and disease‐free survival (DFS) were significantly higher in AML than in ALL, 43% versus 13% and 68% versus 0% respectively. Among the congenital AML cases, six spontaneous remissions have been described. In conclusion, the clinical characteristics of congenital leukaemia differ from those of leukaemia in older children and prognosis is generally poor. Once complete remission is achieved, patients with AML fare better than those with ALL. Chemotherapy for congenital leukaemia needs improvement to increase the sustained remission rate.
ISSN:0007-1048
1365-2141
DOI:10.1046/j.1365-2141.2002.03459.x