Aberrant myelomonocytic CD56 expression in Down syndrome is frequent and not associated with leukemogenesis
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2021 |
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| Erschienen: |
2021 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:100 |
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| Enthalten in: |
Annals of hematology - 100(2021), 7 vom: 27. Juli, Seite 1695-1700 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Gadgeel, Manisha [VerfasserIn] |
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| Links: |
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| Anmerkungen: |
Date Completed 21.06.2021 Date Revised 21.06.2021 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1007/s00277-021-04531-x |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM324439628 |
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| 100 | 1 | |a Gadgeel, Manisha |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Aberrant myelomonocytic CD56 expression in Down syndrome is frequent and not associated with leukemogenesis |
| 264 | 1 | |c 2021 | |
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| 500 | |a Date Completed 21.06.2021 | ||
| 500 | |a Date Revised 21.06.2021 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Aberrant CD56 expression | |
| 650 | 4 | |a Down syndrome | |
| 650 | 4 | |a Immunophenotyping | |
| 650 | 4 | |a Neural adhesion molecule 1 | |
| 650 | 4 | |a Transient abnormal myelopoiesis | |
| 650 | 7 | |a Antigens, CD |2 NLM | |
| 650 | 7 | |a CD56 Antigen |2 NLM | |
| 650 | 7 | |a Core Binding Factor Alpha 2 Subunit |2 NLM | |
| 650 | 7 | |a NCAM1 protein, human |2 NLM | |
| 650 | 7 | |a RUNX1 protein, human |2 NLM | |
| 700 | 1 | |a AlQanber, Batool |e verfasserin |4 aut | |
| 700 | 1 | |a Buck, Steven |e verfasserin |4 aut | |
| 700 | 1 | |a Taub, Jeffrey W |e verfasserin |4 aut | |
| 700 | 1 | |a Ravindranath, Yaddanapudi |e verfasserin |4 aut | |
| 700 | 1 | |a Savaşan, Süreyya |e verfasserin |4 aut | |
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