Emergency: requires immediate attention
Congenital leukemia
Alerts and Notices
Important News & Links
Synopsis
Congenital leukemia refers to leukemia that presents at birth or during the first month of life. However, because numerous cases of leukemia appearing later in infancy have been linked to chromosomal rearrangements that occurred in utero, most, if not all, cases of infant leukemia are now thought to be congenital in origin. Leukemias in the neonate and infant have different clinical and biologic features than those of older children. Acute myelocytic leukemia (AML) is more common than acute lymphocytic leukemia (ALL) in the neonate, in contradistinction to older children; acute megakaryocytic leukemia (AMKL) and acute erythroblastic leukemia occur less commonly. There is an equal incidence among males and females, though females with ALL have a markedly worse prognosis. Maternal alcohol consumption has been associated with increased risk of infant leukemia, particularly AML.
The characteristic clinical presentation of all subtypes in the neonatal period is leukemia cutis and hepatosplenomegaly, with a majority of patients also demonstrating hyperleukocytosis and evidence of meningeal involvement. When leukemias present later in infancy, signs and symptoms are often less specific and include low-grade fever, pallor, lethargy, hepatosplenomegaly, bleeding diathesis, diarrhea, or failure to thrive. The prognosis of neonatal / infant leukemia is generally bleak, with or without chemotherapy.
The characteristic clinical presentation of all subtypes in the neonatal period is leukemia cutis and hepatosplenomegaly, with a majority of patients also demonstrating hyperleukocytosis and evidence of meningeal involvement. When leukemias present later in infancy, signs and symptoms are often less specific and include low-grade fever, pallor, lethargy, hepatosplenomegaly, bleeding diathesis, diarrhea, or failure to thrive. The prognosis of neonatal / infant leukemia is generally bleak, with or without chemotherapy.
Codes
ICD10CM:
C95.90 – Leukemia, unspecified not having achieved remission
SNOMEDCT:
93143009 – Leukemia, disease
C95.90 – Leukemia, unspecified not having achieved remission
SNOMEDCT:
93143009 – Leukemia, disease
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
To perform a comparison, select diagnoses from the classic differential
Subscription Required
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
References
Subscription Required
Last Updated:02/02/2022
Emergency: requires immediate attention
Congenital leukemia