Transient myeloproliferative disorder associated with trisomy 21: Is a short course of chemotherapy indicated in patients with liver impairment and severe clinical problems?

Rizzari, Carmelo; R, Malberti; Dell'Orto, Mariagrazia; Milani, Marina; Jankovic, Momcilo; Ferrari, Erica; Conter, Valentino

doi:10.1002/(sici)1096-911x(199906)32:6<453::aid-mpo14>3.0.co;2-x

Cited by 9 publications

(5 citation statements)

References 11 publications

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“…The present case developed cardiorespiratory insufficiency in association with hyperviscosity syndrome. Cytoreductive therapy, including exchange transfusion or cytotoxic agents, may be the therapy for the prominent increase of the blasts 7 . Because chemotherapy and exchange transfusion appeared risky, the present patient only received supportive therapy.…”

Section: Discussionmentioning

confidence: 93%

Tumor cell lysis syndrome resulting from transient abnormal myelopoiesis in a neonate with Down’s syndrome

Kato

Hoshino²,

Kawataki³

et al. 2001

Pediatrics International

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Newborns with Down's syndrome occasionally present with transient proliferation of blasts, designated as transient abnormal myelopoiesis (TAM) 1 or transient myeloproliferative disorder. The blasts have monoclonality and a megakaryocyte nature with the capacity to differentiate towards multilineage blood cells. 2 The disorder is assumed to be different from 'true malignancy', because the disease regresses spontaneously. However several complications, including hyperviscosity syndrome, hemorrhage and liver fibrosis, have been described and the prognosis is not always favorable. [3][4][5] Acute tumor cell lysis syndrome (ATLS) is a metabolic disorder characterized by hyperkalemia, hyperuricemia and hyperphosphatemia, which results from rapid degradation of malignant cells after initiation of effective chemotherapy. 6 In the present study, we report on a case with TAM, who developed ATLS without any cytotoxic therapies.

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Section: Discussionmentioning

confidence: 93%

Tumor cell lysis syndrome resulting from transient abnormal myelopoiesis in a neonate with Down’s syndrome

Kato

Hoshino²,

Kawataki³

et al. 2001

Pediatrics International

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“…Two treatment modalities, including cytarabine and ExT or leukapheresis (ExT/L) using either alone or both, have been reported as active treatments, and cytarabine was reported to be effective for avoiding early death in TL patients . Although the safety and beneficial effect of ExT on cytoreduction for leukemia patients with hyperleukocytosis have been demonstrated, showing improvement of respiratory symptoms attributable to leukostasis in some cases, its effect on TL patients has not been investigated.…”

Section: Discussionmentioning

confidence: 99%

Exchange transfusion in patients with Down syndrome and severe transient leukemia

Hayasaka

Cho

Morioka

et al. 2015

Pediatrics International

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“…In spite of a self-limiting disease, TAM does not always have favorable clinical course; hyperleukocytosis causes hyperviscosity syndrome leading to cardiorespiratory failure; and humoral mediator derived from blasts induces liver fibrosis leading to hepatic failure [14][15][16]. Given these fatal complications, it is relevant to start chemotherapy in a case with TAM who develops severe such complications [16].…”

Section: Discussionmentioning

confidence: 99%

Transient abnormal myelopoiesis in a cytogenetically normal neonate

et al. 2010

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We present a cytogenetically normal neonate who developed transient abnormal myelopoiesis. The blasts showed trisomy 21. In contrast, fibroblasts, and PHA-stimulated peripheral blood demonstrated normal diploid line on extensive karyotyping. Direct sequencing of the DNA derived from the peripheral blood at overt disease revealed splice site mutation in the boundary of GATA1 exon 2. The patient received three courses of chemotherapy leading to complete remission. During the complete remission, there was neither mutation of GATA1 exon 2 nor trisomy 21, confirming somatic nature of both abnormalities. The patient is now free from the disease 12 months after remission. This case emphasizes the significance of trisomy 21 as the cause of transient abnormal myelopoiesis in Down syndrome.

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Transient myeloproliferative disorder associated with trisomy 21: Is a short course of chemotherapy indicated in patients with liver impairment and severe clinical problems?

Cited by 9 publications

References 11 publications

Tumor cell lysis syndrome resulting from transient abnormal myelopoiesis in a neonate with Down’s syndrome

Tumor cell lysis syndrome resulting from transient abnormal myelopoiesis in a neonate with Down’s syndrome

Exchange transfusion in patients with Down syndrome and severe transient leukemia

Transient abnormal myelopoiesis in a cytogenetically normal neonate

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