Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Correspondence
  • Published:

Spontaneous remission from RAEB in a child

Leukemia volume 15, pages 856–857 (2001)Cite this article

The term myelodysplastic syndromes (MDS) includes a group of hematologic disorders characterized by cytopenia of various degrees, in combination with a hypercellular bone marrow showing dysplastic changes. Current knowledge in this field, as to pediatric cases, lags behind that of childhood acute leukemias as a consequence of many concurrent factors including lower incidence, uncertain classification, difficult diagnosis, variability of the clinical picture, some possibly associated conditions and the lack of consistent cytogenetic abnormalities specific to a single disorder.12 Refractory anemia (RA), RA with excessive blasts (RAEB), RAEB in transformation (RAEB-T), RA with ringed sideroblasts (RARS), and chronic myelomonocytic leukemia (CMML, recently named JMML),3 are the main categories of MDS as set out in the French–American–British (FAB) classification.4 RAEB accounts for about one quarter of all MDS cases. The main features of RAEB are median age at presentation of 7 years, blast cells <5%, dysplasia and cytopenia of two or more cell lines in the peripheral blood, granulocytic or erythroid hyperplasia, dysplasia of myeloid, erythroid and/or megakaryocytic cell lines in the bone marrow.124

Standard treatment of childhood RAEB remains controversial. Most patients progress to develop acute leukemia.2 In such patients intensive chemotherapy has been less successful than in acute myeloid leukemia because of refractory disease, as well as a high rate of toxic death, related to more prolonged cytopenia.2 Therefore, myeloablative therapy supported by a bone marrow transplant may be necessary.56 Nevertheless, sporadic reports of spontaneous remission may challenge this approach. We report a long lasting spontaneous remission from RAEB in an infant.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

References

  1. Fenaux P, Morel P, Lai-JL . Cytogenetics of myelodysplastic syndromes Semin Hematol 1996 33: 127–138

    CAS  PubMed  Google Scholar 

  2. Hasle H . Myelodysplastic syndromes in childhood – classification, epidemiology, and treatment Leuk Lymphoma 1994 13: 11–26

    Article  CAS  Google Scholar 

  3. Niemeyer C, Aricò M, Biondi A, Basso G, Cantù Rajnoldi A, Creutzig U, Haas O, Harbott J, Hasle H, Kerndrup G, Locatelli F, Mann G, Stollmann-Gibbels B, van't Veer-Korhof E, vanWering E, Zimmermann M, and members of the European Working group on Myelodysplastic Syndromes in Childhood (EWOG-MDS) . Chronic myelomonocytic leukemia in childhood: a retrospective analysis of 110 cases Blood 1997 89: 3534–3543

    CAS  PubMed  Google Scholar 

  4. Kouides PA, Bennett JM . Morphology and classification of the myelodysplastic syndromes and their pathologic variants Semin Hematol 1996 33: 95–110

    CAS  PubMed  Google Scholar 

  5. De Witte T, Zwaan F, Hermans J, Vernant J, Kolb H, Vossen J, Lonnqvist B, Beelen D, Ferrant A, Gmur J, Liu Yin J, Troussard X, Cahn J, Van Lint M, Gratwohl A for the Working Party on Leukemia, European Cooperative Group for Bone Marrow Transplantation . Allogeneic bone marrow transplanatation for secondary leukemia and myelodysplastic syndrome. A survey by the Leukemia Working Party and the European Bone Marrow Transplantation Group (EBMTG) Br J Haematol 1990 74: 151–155

    Article  CAS  Google Scholar 

  6. Anderson JE, Anasetti C, Appelbaum FR, Schoch G, Gooley TA, Hansen JA, Buckner CD, Sanders JE, Sullivan KM, Storb R . Unrelated donor marrow transplantation for myelodysplasia (MDS) and MDS-related acute myeloid leukaemia Br J Haematol 1996 93: 59–67

    Article  CAS  Google Scholar 

  7. Dervenoulas JG, Tsirigotis P, Bollas G, Pappa V, Raptis S . Spontaneous remission in myelodysplastic syndrome Ann Hematol 1999 78: 89–90

    Article  CAS  Google Scholar 

  8. Deharo S, Bizet M, Monconduit M, Tilly H, Sari F, Piguet H . Spontaneous remission of refractory anemia with excess of blasts after septicemia Ann Med Intern 1987 138: 663–664

    CAS  Google Scholar 

  9. Broun ER, Heerema NA, Tricot G . Spontaneous remission in myelodysplastic syndrome. A case report Cancer Genet Cytogenet 1990 46: 125–128

    Article  CAS  Google Scholar 

  10. Scheurlen W, Borkhardt A, Ritterbach J, Huppertz HI . Spontaneous hematological remission in a boy with myelodysplastic syndrome and monosomy 7 Leukemia 1994 8: 1435–1438

    CAS  PubMed  Google Scholar 

  11. Bader-Meunier B, Thollot F, Mielot F, Yvart J, Dommergues JP, Sommelet D, Tchernia G . Regressive myelodysplastic syndromes in children. Committee on Childhood Myelodysplasia of the Society of Pediatric Hematology and Immunology Arch Fr Pediatr 1992 49: 883–886

    CAS  PubMed  Google Scholar 

  12. Renneboog B, Hansen V, Heimann P, De Mulder A, Jannsen F, Ferster A . Spontaneous remission in a patient with therapy-related myelodysplastic syndrome (t-MDS) with monosomy 7 Br J Haematol 1996 92: 696–698

    Article  CAS  Google Scholar 

Download references

Acknowledgements

We are indebted to Dr E Percivalle for virologic studies.

Author information

Authors and Affiliations

  1. Dipartimento di Pediatria, II Università di Napoli, Italy

    A De Simone & M D'Avanzo

  2. Laboratorio di Ricerche Cliniche, Anatomia ed Istologia Patologica, Istituti Clinici di Perfezionamento, Milano, Italy

    A Cantù Rajnoldi

  3. Clinica Pediatrica Università di Padova, Italy

    L Sainati

  4. Clinica Pediatrica, IRCCS Policlinico, S Matteo, Pavia, Italy

    M Allen & M Aricò

  5. Dipartimento di Scienze Biomediche Sperimentali e Cliniche, Biologia e Genetica, Università dell'Insubria, Varese, Italy

    E Maserati & F Pasquali

Authors
  1. A De Simone
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. A Cantù Rajnoldi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. L Sainati
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. M D'Avanzo
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. M Allen
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. E Maserati
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. F Pasquali
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. M Aricò
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

De Simone, A., Cantù Rajnoldi, A., Sainati, L. et al. Spontaneous remission from RAEB in a child. Leukemia 15, 856–857 (2001). https://doi.org/10.1038/sj.leu.2402099

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/sj.leu.2402099

Search

Advanced search

Quick links