Leukemia and/or myeloproliferative syndrome in neonates with Down Syndrome

Zipursky, Alvin; Brown, Elizabeth J.; Christensen, Hilary; Sutherland, Robert; Doyle, John

doi:10.1016/s0146-0005(97)80025-0

Cited by 136 publications

(100 citation statements)

References 14 publications

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“…The blast cells often have cell surface antigens characteristic of megakaryoblasts. 21 The condition is referred to as transient abnormal myelopoiesis (TAM), transient leukemic reaction, transient myeloproliferative disorder or transient leukemia. 21 Life-threatening complications may occur in a few patients, but spontaneous remission appears in the majority within 3 months.…”

Section: Transient Abnormal Myelopoiesismentioning

confidence: 99%

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A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases

Hasle

Niemeyer

Chessells³

et al. 2003

Leukemia

393

300

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Myelodysplastic and myeloproliferative disorders are rare in childhood and there is no widely accepted system for their diagnosis and classification. We propose minimal diagnostic criteria and a simple classification scheme which, while based on accepted morphological features and conforming with the recent suggestions of the WHO, allows for the special problems of myelodysplastic diseases in children. The classification recognizes three major diagnostic groups: (1) juvenile myelomonocytic leukemia (JMML), previously named chronic myelomonocytic leukemia (CMML) or juvenile chronic myeloid leukemia (JCML); (2) myeloid leukemia of Down syndrome, a disease with distinct clinical and biological features, encompassing both MDS and AML occurring in Down syndrome; and (3) MDS occurring both de novo and as a complication of previous therapy or pre-existing bone marrow disorder (secondary MDS). The main subtypes of MDS are refractory cytopenia (RC) and refractory anemia with excess of blasts (RAEB). It is suggested retaining the subtype of RAEB-T with 20-30% blasts in the marrow until more data are available. Cytogenetics and serial assessments of the patients are essential adjuncts to morphology both in diagnosis and classification.

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Section: Transient Abnormal Myelopoiesismentioning

confidence: 99%

“…AML develops 1-3 years later in about one quarter of the children. 21 It is uncertain whether TAM should be considered a malignant disease.…”

Section: Transient Abnormal Myelopoiesismentioning

confidence: 99%

A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases

Hasle

Niemeyer

Chessells³

et al. 2003

Leukemia

393

300

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“…Survival of a TL with subsequent development of clonal ALL or myeloid leukemia is reminiscent of leukemogenesis in Down syndrome neonates. 42 Approximately 10% of newborn infants with Down syndrome develop myeloid leukemia/myeloproliferative disease, which usually resolves spontaneously during the first months of life. In approximately 25% of cases that recover, AL will develop in the first 4 years of life.…”

Section: Hsc Were Targeted By Dtrkamentioning

confidence: 99%

Remarkable leukemogenic potency and quality of a constitutively active neurotrophin receptor, ΔTrkA

et al. 2007

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Neurotrophins and their receptors play a key role in neurogenesis and survival. However, we and others have recently obtained evidence for a potential involvement of this receptor system in leukemia. To investigate mechanisms underlying the leukemogenic potential of activated neurotrophin receptor signaling, we analyzed in vivo leukemogenesis mediated by DTrkA, a mutant of TRKA (tropomyosin-related kinase A) isolated from a patient with acute myeloid leukemia (AML). Retroviral expression of DTrkA in myeloid 32D cells induced AML in syngeneic C3H/Hej mice (n ¼ 11/11, latency B4 weeks). C57Bl/6J mice transplanted with DTrkA-transduced primary lineage negative (Lin À ) bone marrow cells died of a transient polyclonal AML (n ¼ 7/15, latency of o12 days). Serial transplantation of AML cells did not re-induce this disease but rather acute lymphoblastic leukemia (ALL, latency 478 days). All primary recipients surviving the early AML developed clonal ALL or myeloid leukemia (latency 472 days) that required additional genetic lesions. PI3K and mTOR-raptor were identified as the crucial mediators of leukemic transformation, whereas STAT and MAP kinase signaling pathways were not activated. Thus, our findings reveal potent and unique transforming properties of altered neurotrophin receptor signaling in leukemogenesis, and encourage further analyses of neurotrophin receptors and downstream signaling events in hematological malignancies.

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“…We are unsure of the reason regarding the difference in myelodysplasia rates but suspect that a part of this variation may be due to: (1) This large significant clinic population, (2) lack of a complete blood count at birth in older patients and (3) potential unintentional bias in the literature due to reports from oncology centres [26].…”

Section: Comorbiditiesmentioning

confidence: 99%

Pediatric Comorbidities and Medical Complications Identified in Children with Down Syndrome

Hickey¹,

Wolter‐Warmerdam²,

Hickey³

et al. 2017

J Down Syndr Chr Abnorm

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Objective: Children with Down syndrome (DS) have an increased risk of neonatal complications and comorbidities compared to the general population; however, the incidence, optimal screening intervals and outcomes in this population are uncertain. The purpose of this study is to phenotypically define this population of children with DS using a large multi-age and ethnic sample and current American Academy of Pediatrics (AAP) Guidelines testing practices.Method: This is a retrospective review of a large cohort of 1,108 children with DS (male=602; mean age at initial contact: 5.72 years, SD ± 5.51) who received care at the Sie Center for Down Syndrome (SCDS) at The Children's Hospital Colorado from 2011 and 2016. Clinical data were collected from a prospective patient clinic database. Clinical details were collected, which included demographics, prenatal and birth history and complications, comorbidities, procedures, treatment plans and outcomes.Results: Medical complications requiring admission to the neonatal intensive care unit were identified in 70.6% of children with DS. Frequent causes for these neonatal admissions included: Required oxygen (60.7%), feeding problem (48.2%), Respiratory Distress Syndrome (21.4%) and pulmonary hypertension (14.7%). Incidence of medical comorbidities in our population with DS included cardiac defects (64.3%), abnormal sleep study (71.1%), abnormal thyroid study (29.1%), pulmonary aspiration (12.2%), celiac disease (5.0%) and pulmonary arterial hypertension (28.3%). Conclusion:Clinical data provides results from one of the largest investigations at a single pediatric hospital for children and young adults with DS in the US. This study describes the comorbidities affecting individuals with DS more accurately by applying AAP guidelines in studying a larger population than previously defined. This result improves our understanding of the incidence and identification of medical conditions in children with DS and reinforces recommendations on medical care screening for individuals with DS.

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Leukemia and/or myeloproliferative syndrome in neonates with Down Syndrome

Cited by 136 publications

References 14 publications

A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases

A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases

Remarkable leukemogenic potency and quality of a constitutively active neurotrophin receptor, ΔTrkA

Pediatric Comorbidities and Medical Complications Identified in Children with Down Syndrome

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