Transient Myeloproliferative Disorder and Eosinophilic Pericardial Effusion in a Down Syndrome Neonate

Shenoy, Rajgopal; Bhat, Kamalakshi G.; Kamath, Nutan; Kumble, Yusuf

doi:10.1080/08880010801888220

Cited by 10 publications

(21 citation statements)

References 17 publications

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“…The various prognostic factors and controversies about treatment indications have been extensively studied, however, literature search shows that only 11 cases have been reported from India [5][6][7][8][9][10][11][12]. The first case of TMD from India is reported in 1996 [5].…”

Section: Discussionmentioning

confidence: 98%

Transient Leukemia in Down Syndrome: Report of Two Cases with Review of Literature

Gosavi

Murarkar

Lanjewar

et al. 2011

Indian J Hematol Blood Transfus

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Transient leukemia (TL) also referred to as transient abnormal myelopoiesis (TAM) or transient myeloproliferative disorder (TMD) is a unique syndrome that frequently occurs in newborns with Down syndrome (DS). It manifests in the first few days of life and shows leukocytosis with blast cells in the blood and bone marrow. This leukemia resolves spontaneously within first few months of life in the majority of cases. In this report we describe two newborns with a karyotype of 47,XY,+21, presented with marked leukocytosis and many blast cells in the peripheral blood. In both the cases, the blasts disappeared and the total leukocyte count reverted to normal without any specific treatment.

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Section: Discussionmentioning

confidence: 98%

Transient Leukemia in Down Syndrome: Report of Two Cases with Review of Literature

Gosavi

Murarkar

Lanjewar

et al. 2011

Indian J Hematol Blood Transfus

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“…17 Eosinophilic pericardial effusion was also documented in a neonate with Down syndrome. 18 The proform of eosinophil major basic protein has been identified as a maternal serum marker for Down syndrome. 19 consists of seven exons, of which exons 1 to 4 can be alternatively transcribed.…”

Section: Mice Bone Marrow Cells From Rcan1mentioning

confidence: 99%

Regulator of Calcineurin 1 (Rcan1) Is Required for the Development of Pulmonary Eosinophilia in Allergic Inflammation in Mice

Yang

MacNeil

Junkins

et al. 2011

The American Journal of Pathology

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The presence of eosinophils in the lung is often regarded as a defining feature of asthma. On allergen stimulation, numbers of eosinophils and their progenitors are increased in both the bone marrow and lungs. Eosinophil progenitors provide an ongoing supply of mature eosinophils. Here, we report that deficiency in the regulator of calcineurin 1 gene (Rcan1) leads to a near-complete absence of eosinophilia in ovalbumin-induced allergic asthma in mice. In the absence of Rcan1, bone marrow cells produce significantly fewer eosinophils in vivo and in vitro on interleukin-5 stimulation. Importantly, eosinophil progenitor populations are significantly reduced in both naïve and ovalbumin-challenged Rcan1 mice. Bone marrow cells from Rcan1؊/؊ mice are capable of developing into fully mature eosinophils, suggesting that Rcan1 is required for eosinophil progenitor production but may not be necessary for eosinophil maturation. Thus, Rcan1 represents a novel contributor in the development of eosinophilia in allergic asthma through regulation of eosinophil progenitor production.

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“…There have been some case reports on DS with pericardial effusion (Kusanagi et al 1998;Hirashima et al 2000;Shenoy et al 2008;Buyukkale et al 2012). Kusanagi et al (1998) reported a DS patient who had hypereosinophilic syndrome, TAM and pericardial effusion.…”

Section: Discussionmentioning

confidence: 99%

“…The patient had 49% blast cells of peripheral white blood cells, but they did not mention about eosinophils. Shenoy et al (2008) reported a patient who had TAM and eosinophilic pericardial effusion. They found 29% eosinophils but no blasts in her pericardial effusion fluid, and her effusion was controlled by administration of prednisolone.…”

Section: Discussionmentioning

confidence: 99%

Cytokine Profiles in Pericardial Effusion in a Down Syndrome Infant with Transient Abnormal Myelopoiesis

Shitara

Takahashi

Aoki

et al. 2017

Tohoku J. Exp. Med.

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Infants with Down Syndrome (DS) are at risk of developing a transient abnormal myelopoiesis (TAM). TAM is characterised by increased circulating blast cells but usually self-limiting. DS patients with TAM sometimes show fetal hydrops and effusion in body cavities, but the mechanism remains unclear. We report here a case of infant with DS who had pericardial effusion, TAM, and eosinophilia. In her pericardial effusion, white blood cell count was 6.0 × 10 3 /µL, 41% of which were eosinophils. After administration of prednisolone, pericardial effusion gradually decreased, and TAM and eosinophilia improved. In order to elucidate the immunological mechanism, we measured the levels of 17 cytokines in her pericardial effusion fluid and serum. In her pericardial fluid, there were high levels of 12 cytokines, and they were higher than those in her serum. In particular, IL-6 (44,573 pg/mL), IL-8 (4,865 pg/mL), and IL-13 (579.41 pg/mL) were at extremely high levels in her pericardial fluid. After administration of prednisolone, the levels of 8 of the 12 elevated cytokines in her pericardial fluid decreased and all of the elevated cytokines decreased in her serum. Corticosteroids can be effective to reduce cytokine levels and the amount of effusion in patients with DS. It is presumed that effusion seen in DS with TAM could be related to an abnormal production of cytokines at the effusion site.

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Transient Myeloproliferative Disorder and Eosinophilic Pericardial Effusion in a Down Syndrome Neonate

Cited by 10 publications

References 17 publications

Transient Leukemia in Down Syndrome: Report of Two Cases with Review of Literature

Transient Leukemia in Down Syndrome: Report of Two Cases with Review of Literature

Regulator of Calcineurin 1 (Rcan1) Is Required for the Development of Pulmonary Eosinophilia in Allergic Inflammation in Mice

Cytokine Profiles in Pericardial Effusion in a Down Syndrome Infant with Transient Abnormal Myelopoiesis

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