Life-threatening complications of transient abnormal myelopoiesis in neonates with Down syndrome (original) (raw)

An Erratum to this article was published on 15 February 2005

Abstract

Neonates with Down syndrome can present with a haematological disorder called transient abnormal myelopoiesis (TAM). While TAM is usually a self-limiting disease, patients with severe complications such as hydrops fetalis, cardiorespiratory failure and liver fibrosis have been described. Here, we present five consecutive neonates with trisomy 21 and TAM, four of whom were critically ill and were therefore treated with cytosine-arabinoside. All five patients survived. _Conclusion:_severely affected neonates with Down syndrome and transient abnormal myelopoiesis might benefit from early cytostatic treatment with cytosine-arabinoside.

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Abbreviations

Ara-C :

cytosine-arabinoside

DS :

Down syndrome

TAM :

transient abnormal myelopoiesis

References

  1. Ahmed M, Sternberg A, Hall G, Thomas A, Smith O, O’Marcaigh A, Wynn R, Stevens R, Addison M, King D, Stewart B, Gibson B, Roberts I, Vyas P (2004) Natural history of GATA 1 mutations in Down syndrome. Blood 103: 2480–2489
    Article PubMed Google Scholar
  2. Al−Kasim F, Doyle JJ, Massey GV, Weinstein HJ, Zipursky A (2002) Incidence and treatment of potentially lethal diseases in transient leukemia of Down syndrome: Pediatric Oncology Group Study. J Pediatr Hematol Oncol 24: 9−13
    Article PubMed Google Scholar
  3. Avet−Loiseau H, Mechinaud F, Harousseau JL (1995) Clonal hematologic disorders in Down syndrome. J Pediatr Hematol Oncol 17: 19–24
    CAS PubMed Google Scholar
  4. Bonno M, Azuma E, Kawasaki H, Zhang ZL, Komada Y, Hirayama M, Higashiwaga M, Umemoto M, Umemoto M, Koike T, Kato T, Tahara T, Miyazaki H, Sakurai M (1998) Thrombopoietin level is inversely related to blast count, not platelet number, in Down syndrome neonates with transient myeloproliferative disorder. Am J Hematol 58: 267–272
    CAS PubMed Google Scholar
  5. Coulombel L, Derycke M, Villeval JL, Leonard C, Breton−Gorius J, Vial M, Bourgeois P, Tchernia G (1987) Characterization of the blast cell population in two neonates with Down’s syndrome and transient myeloproliferative disorder. Br J Haematol 66: 69–76
    CAS PubMed Google Scholar
  6. Creutzig U, Ritter J, Vormoor J, Eschenbach C, Dickerhoff R, Burdach S, Scheel−Walter HG, Kühl J, Schellong G (1990) Transient myeloproliferation and acute myeloid leukemia in infants with Down’s syndrome. Klin Pädiatr 202: 253–257
    Google Scholar
  7. Garel D, Mielot F, Gaulard P, Quillard J, Doomergues JP, Tchernia G (1991) Acute megakaryocytic leukaemia (AMKL) with major myelofibrosis in an infant. Diagnosis by liver biopsy and response to treatment. Nouv Rev Fr Hematol 33: 5−8
    CAS PubMed Google Scholar
  8. Hirashima C, Eguchi Y, Kohumara Y, Minatami H, Sato I (2000) Isolated pericardial effusion and transient abnormal myelopoiesis in a fetus with Down’s syndrome. J Obstet Gynaecol Res 26: 303–306
    CAS PubMed Google Scholar
  9. Hitzler JK, Cheung J, Li Y, Scherer SW, Zipursky A (2003) GATA 1 mutations in transient leukemia and acute megakaryoblastic leukemia in Down syndrome. Blood 101: 4301–4304
    Article CAS PubMed Google Scholar
  10. Homans AC, Verissimo AM, Vlacha V (1993) Transient abnormal myelopoiesis of infancy associated with trisomy 21. Am J Pediatr Hematol Oncol 15: 392–399
    CAS PubMed Google Scholar
  11. Schwab M, Niemeyer CM, Schwarzer U (1998) Down syndrome, transient myeloproliferative disorder and infantile liver fibrosis. Med Pediatr Oncol 31: 159–165
    Article CAS PubMed Google Scholar
  12. Tchernia G, Lejeune F, Boccara JF, Denavit MF, Dommergues JP, Bernaudin F (1996) Erythroblastic and/or megakaryoblastic leukemia in Down syndrome: treatment with low-dose arabinosyl cytosine. J Pediatr Hematol Oncol 18: 59–62
    Article CAS PubMed Google Scholar
  13. Yagihashi N, Watanabe K, Yagihashi S (1995) Transient abnormal myelopoiesis accompanied by hepatic fibrosis in two infants with Down syndrome. J Clin Pathol 48: 973–975
    Google Scholar
  14. Yoshioka K, Yamamoto S, Moriguchi N, Miyata H, Tsukiyama K, Isokawa S, Horiuchi F, Takemura T (2000) Over-expression of bcl−2 in transient abnormal myelopoiesis associated with Down syndrome. Ann Hematol 79: 319–321
    Article CAS PubMed Google Scholar
  15. Zipursky A (1996) The treatment of children with acute megakaryoblastic leukaemia who have Down syndrome. Pediatr Hematol Oncol 18: 10–12
    Article Google Scholar
  16. Zipursky A, Poon A, Doyle J (1992) Leukemia in Down syndrome: a review. Pediatr Hematol Oncol 9: 139–149
    CAS PubMed Google Scholar
  17. Zipursky A, Brown EJ, Christensen H, Doyle J (1999) Transient myeloproliferative disorder (transient leukemia) and the hematologic manifestations of Down syndrome. Clin Lab Med 19: 157–167
    CAS PubMed Google Scholar

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Authors and Affiliations

  1. Department of Paediatrics and Adolescent Medicine, University of Freiburg, Mathildenstrasse 1, 79106 , Freiburg, Germany
    Sabine Dormann, Marcus Krüger, Roland Hentschel, Brigitte Strahm, Udo Kontny & Charlotte Niemeyer
  2. Department of Obstetrics and Gynaecology, University of Freiburg, Hugstetterstrasse 55, 79106, Freiburg, Germany
    Regina Rasenack

Authors

  1. Sabine Dormann
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  2. Marcus Krüger
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  3. Roland Hentschel
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  4. Regina Rasenack
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  5. Brigitte Strahm
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  6. Udo Kontny
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  7. Charlotte Niemeyer
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Corresponding author

Correspondence toSabine Dormann.

Additional information

An erratum to this article can be found at http://dx.doi.org/10.1007/s00431-004-1617-4

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Dormann, S., Krüger, M., Hentschel, R. et al. Life-threatening complications of transient abnormal myelopoiesis in neonates with Down syndrome.Eur J Pediatr 163, 374–377 (2004). https://doi.org/10.1007/s00431-004-1452-7

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