Primary Myelofibrosis with Myeloid Metaplasia and Cytogenetically Abnormal Clones in 2 Children with Down's Syndrome

Ueda, Kazuhiro; Kawaguchi, Yoshindo; Kodama, Motomu; Tanaka, Yoshito; Usui, Tateo; Kamada, Nanao

doi:10.1111/j.1600-0609.1981.tb00466.x

Cited by 24 publications

(2 citation statements)

References 16 publications

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“…Review of English literature yielded 47 reported cases of childhood idiopathic myelofibrosis [2]. Only six of these cases were associated with DS [2][3][4][5]. In recent times, it has become increasingly recognised that DS (trisomy 21) may predispose to various myeloid proliferations [1].…”

Section: Discussionmentioning

confidence: 99%

Chronic Childhood Idiopathic Myelofibrosis in Down’s Syndrome: A Case Report

Singh

Sharma

Tejwani

et al. 2011

Indian J Hematol Blood Transfus

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Idiopathic myelofibrosis a disease of elderly is rarely seen in children. A case of chronic idiopathic myelofibrosis in an 8 year old boy with Down's syndrome is reported here, who presented with progressive pallor and hepatosplenomegaly. Peripheral blood examination revealed pancytopenia, macrocytic anemia and tear drop cells. No blasts were found. Bone marrow aspirate yielded a dry tap and trephine biopsy showed marrow fibrosis with osteosclerosis. Focally megakaryocytes were increased in number with atypical morphology. No blasts were seen. Review of literature revealed 47 reported cases of childhood idiopathic myelofibrosis. Six cases were associated with Down's syndrome and only 3 of them had features of chronic idiopathic myelofibrosis without evidence of acute megakaryoblastic leukaemia.

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Section: Discussionmentioning

confidence: 99%

Chronic Childhood Idiopathic Myelofibrosis in Down’s Syndrome: A Case Report

Singh

Sharma

Tejwani

et al. 2011

Indian J Hematol Blood Transfus

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“…In these cases, transformation to acute leukemia was seen within months and the prognosis was poor. [20][21][22] In present patient, this transformed to AML-M7 7 months after diagnosis. The patient with underlying congenital heart and chronic lung disease died.…”

Section: Discussionmentioning

confidence: 99%

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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Acute megakaryoblastic leukemia associated with mosaic Down's syndrome

Simon

Tebbi

Freeman

et al. 1987

Cancer

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There have been several reports of the association between Down's Syndrome and acute megakaryoblastic (M7) leukemia (AMKL). The diagnosis of this rare form of leukemia has been better delineated by the use of the platelet peroxidase reaction and the antifactor VIII antibody immunoperoxidase test. In the past, the prognosis of patients with a combination of Down's Syndrome and AMKL has been extremely poor, with a median survival of 6.9 months for 11 reported cases in the literature. The present report reviews the previously reported cases and describes a unique patient with mosaic Down's syndrome and AMKL with favorable response to therapy.

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Primary Myelofibrosis with Myeloid Metaplasia and Cytogenetically Abnormal Clones in 2 Children with Down's Syndrome

Cited by 24 publications

References 16 publications

Chronic Childhood Idiopathic Myelofibrosis in Down’s Syndrome: A Case Report

Chronic Childhood Idiopathic Myelofibrosis in Down’s Syndrome: A Case Report

Evaluation of hematological findings in children with Down syndrome

Acute megakaryoblastic leukemia associated with mosaic Down's syndrome

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