TY - JOUR
T1 - Aberrant myelomonocytic CD56 expression in Down syndrome is frequent and not associated with leukemogenesis
AU - Gadgeel, Manisha
AU - AlQanber, Batool
AU - Buck, Steven
AU - Taub, Jeffrey W.
AU - Ravindranath, Yaddanapudi
AU - Savaşan, Süreyya
N1 - Funding Information:
This study is supported partially by funds from Children’s Foundation and Kids Without Cancer Foundation. J.W.T. is supported by Ring Screw Textron Endowed Chair, and Y.R. is supported by Georgie Ginopolis chair award.
Publisher Copyright:
© 2021, The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.
PY - 2021/7
Y1 - 2021/7
N2 - Children with Down syndrome (DS) are at an increased risk of developing transient abnormal myelopoiesis (TAM) and acute leukemia. Aberrant expression of CD56 has been observed on myeloid leukemic blasts in DS patients. In general, CD56 expression in acute myeloid leukemia (AML) is considered a promoter of leukemogenesis. We did a retrospective flow cytometric study to investigate mature myelomonocytic cell CD56 expression patterns in TAM, non-TAM, and leukemia cases with DS. Flow cytometric analysis showed that granulocyte and monocyte aberrant/dysplastic CD56 expression is an inherent characteristic of most DS patients irrespective of the presence of TAM or leukemia. Increased CD56 expression in monocyte and granulocyte populations in DS could be multifactorial; greater expression of RUNX1 secondary to the gene dose effect of trisomy 21 along with the maturational state of the cells are the potential contributors. Unlike AML seen in non-DS patients, CD56 overexpression in DS AML cases does not appear to play a role in leukemogenesis.
AB - Children with Down syndrome (DS) are at an increased risk of developing transient abnormal myelopoiesis (TAM) and acute leukemia. Aberrant expression of CD56 has been observed on myeloid leukemic blasts in DS patients. In general, CD56 expression in acute myeloid leukemia (AML) is considered a promoter of leukemogenesis. We did a retrospective flow cytometric study to investigate mature myelomonocytic cell CD56 expression patterns in TAM, non-TAM, and leukemia cases with DS. Flow cytometric analysis showed that granulocyte and monocyte aberrant/dysplastic CD56 expression is an inherent characteristic of most DS patients irrespective of the presence of TAM or leukemia. Increased CD56 expression in monocyte and granulocyte populations in DS could be multifactorial; greater expression of RUNX1 secondary to the gene dose effect of trisomy 21 along with the maturational state of the cells are the potential contributors. Unlike AML seen in non-DS patients, CD56 overexpression in DS AML cases does not appear to play a role in leukemogenesis.
KW - Aberrant CD56 expression
KW - Down syndrome
KW - Immunophenotyping
KW - Neural adhesion molecule 1
KW - Transient abnormal myelopoiesis
UR - http://www.scopus.com/inward/record.url?scp=85104674002&partnerID=8YFLogxK
U2 - 10.1007/s00277-021-04531-x
DO - 10.1007/s00277-021-04531-x
M3 - Article
C2 - 33890142
AN - SCOPUS:85104674002
VL - 100
SP - 1695
EP - 1700
JO - Annals of Hematology
JF - Annals of Hematology
SN - 0939-5555
IS - 7
ER -