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MONITORING OF TRANSIENT MYELOPROLIFERATIVE DISORDER AND LEUKEMIA IN DOWN’S SYNDROME : A SINGLE UNIVERSITY HOSPITAL STUDY
Author(s): ,
Jun Eun Park
Affiliations:
Pediatrics,AJOU UNIVERSITY SCHOOL OF MEDICINE,Suwon,Korea, Republic Of
Inchan Hwang
Affiliations:
Pediatrics,AJOU UNIVERSITY SCHOOL OF MEDICINE,Suwon,Korea, Republic Of
(Abstract release date: 05/18/17) EHA Library. Eun Park J. 05/18/17; 182772; PB2058
Jun Eun Park
Jun Eun Park
Contributions
Abstract

Abstract: PB2058

Type: Publication Only

Background
Children with Down syndrome (DS) have a 10- to 20-fold increased risk of developing leukemia. But some patients don’t suffer leukemia even they have significant numbers of blast cell in their peripheral blood. These condition called Transient myeloproliferative disoder(TMD), and it is a disease entity unique to DS newborns and is defined as the morphologic detection of blasts in DS less than three months of age.

Aims
This study gathered DS patients to find some difference between leukemia and TMD, to determine prognosis and risk factors.

Methods
We collect 317 patient’s blood lab results in 433 DS patients. 102 patients has leukocytosis, and in 18 case found blast cells in their peripheral blood.

Results
12 patients have found blast in three months of life, 11 of them finally diagnosed to TMD, and only 1 patient progress to Acute Myeloid Leukemia(AML) in 98 days of his life. Other 6 patients have blast in their blood after three months of life, and underwent chemotherapy due to hematologic malignancy. All patients with leukemia has anemia at diagnosis, which is not found in TMD patients(p=0.018). In 7 Leukemia patients, 3 was acute Lymphoblastic Leukemia(ALL), 4 was AML. All AML patients has clonal change additional to trisomy 21 at their diagnostic point, which didn’t found at TMD and ALL patients, even it didn’t confirm former examination.

Conclusion
DS Patient who has blast in their peripheral blood before 3 months of life need closely follow up their Complete Blood Count and Chromosome analysis to find whether TMD progress to leukemia.

Session topic: 16. Myeloproliferative neoplasms - Clinical

Keyword(s): Myeloproliferative disorder, Down Syndrome

Abstract: PB2058

Type: Publication Only

Background
Children with Down syndrome (DS) have a 10- to 20-fold increased risk of developing leukemia. But some patients don’t suffer leukemia even they have significant numbers of blast cell in their peripheral blood. These condition called Transient myeloproliferative disoder(TMD), and it is a disease entity unique to DS newborns and is defined as the morphologic detection of blasts in DS less than three months of age.

Aims
This study gathered DS patients to find some difference between leukemia and TMD, to determine prognosis and risk factors.

Methods
We collect 317 patient’s blood lab results in 433 DS patients. 102 patients has leukocytosis, and in 18 case found blast cells in their peripheral blood.

Results
12 patients have found blast in three months of life, 11 of them finally diagnosed to TMD, and only 1 patient progress to Acute Myeloid Leukemia(AML) in 98 days of his life. Other 6 patients have blast in their blood after three months of life, and underwent chemotherapy due to hematologic malignancy. All patients with leukemia has anemia at diagnosis, which is not found in TMD patients(p=0.018). In 7 Leukemia patients, 3 was acute Lymphoblastic Leukemia(ALL), 4 was AML. All AML patients has clonal change additional to trisomy 21 at their diagnostic point, which didn’t found at TMD and ALL patients, even it didn’t confirm former examination.

Conclusion
DS Patient who has blast in their peripheral blood before 3 months of life need closely follow up their Complete Blood Count and Chromosome analysis to find whether TMD progress to leukemia.

Session topic: 16. Myeloproliferative neoplasms - Clinical

Keyword(s): Myeloproliferative disorder, Down Syndrome

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