Transient abnormal myelopoiesis

This disease is not reportable.
Name
Transient abnormal myelopoiesis
ICD-O-3 Morphology
9898/1
Effective 2001 and later
Reportable
This disease is not reportable
Primary Site(s)
C421
Alternate Names
TAM
Transient myeloproliferative disorder
Definition
Transient abnormal myelopoiesis (TAM) is a unique disorder of Down syndrome newborns that presents with clinical and morphologic findings indistinguishable from AML. The blasts have morphologic and immunologic features of megakaryocytic lineage. This is part of the myeloid proliferations with Down syndrome WHO group, which also includes myeloid leukemia associated with Down syndrome (see 9898/3).
Definitive Diagnostic Methods
Clinical
Immunophenotyping
Genetics Data
GATA1
Trisomy 21
Immunophenotyping
Negative: Myeloperoxidase, CD15, CD14, Glycophorin A
Positive: CD4 dim, CD7, CD13, CD117, CD33, CD34, CD36, CD42, CD61, CD71, CD56, CD41, HLA-DR + (approx 30% of cases), TPO-R, IL-3R
Treatments
No treatments found
Corresponding ICD-9 Codes
238.79 Other lymphatic and hematopoietic tissues
Corresponding ICD-10 Codes
D46.7 Other myelodysplastic syndromes
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
D46.Z Other myelodysplastic syndrome