Risks of leukaemia and solid tumours in individuals with Down's syndrome

Hasle, Henrik; Clemmensen, Inge; Mikkelsen, Margareta

doi:10.1016/s0140-6736(99)05264-2

Cited by 741 publications

(666 citation statements)

References 35 publications

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“…The prognosis was found to be worse than sporadic lymphoid leukemia patients due to intrinsic drug resistance and treatment-related mortality. 13,14 In present cases, only 1 patient developed ALL. In the consolidation treatment of the patient, severe mucositis, elevation of liver function tests and kidney failure developed with methotrexate.…”

Section: Discussionmentioning

confidence: 44%

Evaluation of hematological findings in children with Down syndrome

Sarbay¹

2018

Int J Contemp Pediatr

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Background: Various hematological disorders and malignancies may be encountered with Down syndrome. In this article, hematological findings of children with Down syndrome were investigated. Methods: The hematological results of 106 Down syndrome children aged 3 months to 17 years were assessed retrospectively. The results were compared with a patient group consisting of non-DS and healthy children with a similar age range. Results: When compared with the control group, a significant difference was found between the frequencies of neutropenia, monocytosis, macrocytosis and anisocytosis. No significant difference was detected between the patients with macrocytosis and those without macrocytosis when the relation of macrocytosis with other hematological disorders was examined. On the other hand, the comparison between the groups with and without macrocytosis showed that the prevalence of acute leukemia was significantly higher in patients with Down syndrome. Conclusions: DS children with macrocytosis should be more closely monitored for myelodysplastic syndrome and acute leukemia.

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Section: Discussionmentioning

confidence: 44%

Evaluation of hematological findings in children with Down syndrome

Sarbay¹

2018

Int J Contemp Pediatr

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“…It also found a difference in organ site and histology distribution of solid neoplasms. 1 In adults this was confirmed in 3 subsequent epidemiologic studies on cancer incidence and on causes of death in DS conducted in Denmark, 2 Israel 3 and the United States, 4 showing a very low incidence of carcinomas while germ cell tumours were more frequent than in the general population. However, for children Ͻ15 years of age, these studies could not provide sufficient data.…”

Section: Dear Sirmentioning

confidence: 79%

Are solid tumours different in children with Down's syndrome?

Satgé¹,

Sasco²,

Lacour³

2003

Intl Journal of Cancer

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confidence: 99%

“…Death from malignancies other than leukemia is strikingly less common in patients of all ages in DS, and death from leukemia is more likely in children younger than 10 years of age with DS than in those without [5]. The most frequent form of leukemia during childhood, both with and without DS, is acute lymphoblastic leukemia (ALL), and the incidence of ALL in children with DS is approximately 20-fold higher than in non-DS children [3]. However, the most marked increase in incidence in DS infants is with acute megakaryoblastic leukemia (AMKL), a myeloid malignancy of platelet precursors.…”

mentioning

confidence: 99%

“…Patients with DS show a unique spectrum of malignancies, with a 10-to 20-fold higher risk of acute leukemia and a lower incidence of solid tumors [3,4]. Death from malignancies other than leukemia is strikingly less common in patients of all ages in DS, and death from leukemia is more likely in children younger than 10 years of age with DS than in those without [5].…”

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confidence: 99%

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Evolution of myeloid leukemia in children with Down syndrome

Saida

2016

Int J Hematol

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the general population, the distribution of malignancies is different [3]. Patients with DS show a unique spectrum of malignancies, with a 10-to 20-fold higher risk of acute leukemia and a lower incidence of solid tumors [3,4]. Death from malignancies other than leukemia is strikingly less common in patients of all ages in DS, and death from leukemia is more likely in children younger than 10 years of age with DS than in those without [5]. The most frequent form of leukemia during childhood, both with and without DS, is acute lymphoblastic leukemia (ALL), and the incidence of ALL in children with DS is approximately 20-fold higher than in non-DS children [3]. However, the most marked increase in incidence in DS infants is with acute megakaryoblastic leukemia (AMKL), a myeloid malignancy of platelet precursors. The relative risk of developing AMKL is estimated to be 500 times higher in children with DS than in the general population [6]. Unlike AMKL in non-DS patients, most AMKL associated with DS (DS-AMKL) patients usually respond well to chemotherapy.In most cases, DS-AMKL is preceded by a temporary form of megakaryoblastic leukemia unique to newborns with DS and known as transient abnormal myelopoiesis (TAM) or transient leukemia. TAM, a clonal myeloproliferative syndrome, presents in the fetus or a few days after birth and in most cases resolves spontaneously within 3 months [7,8]. Typically, TAM is characterized by the presence of high numbers of circulating blast cells expressing CD33, CD38, CD117, CD34, CD7, CD56, CD36, CD71, and CD42b [9], which are indistinguishable from blasts observed in DS-AMKL. More often, the clinical presentation of TAM varies from asymptomatic alterations in the blood count to disseminated leukemic infiltration. Clinically severe TAM (liver failure, pleural/pericardial effusion, ascites, renal failure, and coagulopathy) affects 10-30 % of patients with clinically diagnosed TAM, and Abstract Children with Down syndrome (DS) have a markedly increased risk of leukemia. They are at particular risk of acute megakaryoblastic leukemia, known as myeloid leukemia associated with DS (ML-DS), the development of which is closely linked to a preceding temporary form of neonatal leukemia called transient abnormal myelopoiesis (TAM). Findings from recent clinical and laboratory studies suggest that constitutional trisomy 21 and GATA1 mutation(s) cause TAM, and that additional genetic alteration(s) including those in epigenetic regulators and signaling molecules are involved in the progression from TAM to ML-DS. Thus, this disease progression represents an important model of multi-step leukemogenesis. The present review focuses on the evolutionary process of TAM to ML-DS, and advances in the understanding of perturbed hematopoiesis in DS with respect to GATA1 mutation and recent findings, including cooperating genetic events, are discussed.

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Risks of leukaemia and solid tumours in individuals with Down's syndrome

Cited by 741 publications

References 35 publications

Evaluation of hematological findings in children with Down syndrome

Evaluation of hematological findings in children with Down syndrome

Are solid tumours different in children with Down's syndrome?

Evolution of myeloid leukemia in children with Down syndrome

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