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Childhood myelodysplastic syndrome

MedGen UID:
389541
•Concept ID:
C2347761
•
Neoplastic Process
Synonyms: childhood MDS; Childhood Myelodysplastic Syndrome; childhood myelodysplastic syndrome
SNOMED CT: Childhood myelodysplastic syndrome (705061009)
 
Monarch Initiative: MONDO:0044873

Definition

An uncommon hematologic malignancy occurring during childhood. Many of the morphologic, immunophenotypic, and genetic changes seen in adult myelodysplastic syndromes are also observed in the childhood variants of the disease. Children present with neutropenia and thrombocytopenia more often than adults, and bone marrow hypocellularity is more often seen in children than adults. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood myelodysplastic syndrome

Professional guidelines

PubMed

Childhood myelodysplastic syndrome: focus on the approach to diagnosis and treatment of juvenile myelomonocytic leukemia.
Loh ML
Hematology Am Soc Hematol Educ Program 2010;2010:357-62. doi: 10.1182/asheducation-2010.1.357. PMID: 21239819
Role of allogeneic bone marrow transplantation for the treatment of myelodysplastic syndromes in childhood. The European Working Group on Childhood Myelodysplastic Syndrome (EWOG-MDS) and the Austria-Germany-Italy (AGI) Bone Marrow Transplantation Registry.
Locatelli F, Zecca M, Niemeyer C, Angelucci E, Arcese G, Bender-Gotze C, Bonetti F, Burdach S, Dini G, Ebell W, Friedrich W, Hasle H, Hermann J, Jacobsen N, Klingebiel T, Kremens B, Mann G, Miniero R, Pession A, Peters C, Paolucci P, Rossetti F, Schmid HJ, Stary J, Zimmermann M
Bone Marrow Transplant 1996 Nov;18 Suppl 2:63-8. PMID: 8932802

Recent clinical studies

Etiology

Wilms' tumor 1 mRNA expression: a good tool for differentiating between myelodysplastic syndrome and aplastic anemia in children?
Zuo Y, Cheng Y, Zhang L, Qin Y, Luo H
Hematology 2019 Dec;24(1):480-486. doi: 10.1080/16078454.2019.1631507. PMID: 31210595
Differences in the bone marrow histology between childhood myelodysplastic syndrome with multilineage dysplasia and refractory cytopenia of childhood without multilineage dysplasia.
Iwafuchi H, Ito M
Histopathology 2019 Jan;74(2):239-247. Epub 2018 Oct 29 doi: 10.1111/his.13721. PMID: 30062702
Childhood myelodysplastic syndrome.
Chatterjee T, Choudhry VP
Indian J Pediatr 2013 Sep;80(9):764-71. Epub 2013 Aug 3 doi: 10.1007/s12098-013-1130-8. PMID: 23912822
Comparison of childhood myelodysplastic syndrome, AML FAB M6 or M7, CCG 2891: report from the Children's Oncology Group.
Barnard DR, Alonzo TA, Gerbing RB, Lange B, Woods WG; Children's Oncology Group
Pediatr Blood Cancer 2007 Jul;49(1):17-22. doi: 10.1002/pbc.20951. PMID: 16856158
The International Prognostic Scoring System (IPSS) for childhood myelodysplastic syndrome (MDS) and juvenile myelomonocytic leukemia (JMML).
Hasle H, Baumann I, Bergsträsser E, Fenu S, Fischer A, Kardos G, Kerndrup G, Locatelli F, Rogge T, Schultz KR, Starý J, Trebo M, van den Heuvel-Eibrink MM, Harbott J, Nöllke P, Niemeyer CM; European Working Group on childhood MDS
Leukemia 2004 Dec;18(12):2008-14. doi: 10.1038/sj.leu.2403489. PMID: 15496981

Diagnosis

Loss of B cells and their precursors is the most constant feature of GATA-2 deficiency in childhood myelodysplastic syndrome.
Nováková M, Žaliová M, Suková M, Wlodarski M, Janda A, Froňková E, Campr V, Lejhancová K, Zapletal O, Pospíšilová D, Černá Z, Kuhn T, Švec P, Pelková V, Zemanová Z, Kerndrup G, van den Heuvel-Eibrink M, van der Velden V, Niemeyer C, Kalina T, Trka J, Starý J, Hrušák O, Mejstříková E
Haematologica 2016 Jun;101(6):707-16. Epub 2016 Mar 24 doi: 10.3324/haematol.2015.137711. PMID: 27013649Free PMC Article
Childhood myelodysplastic syndrome.
Chatterjee T, Choudhry VP
Indian J Pediatr 2013 Sep;80(9):764-71. Epub 2013 Aug 3 doi: 10.1007/s12098-013-1130-8. PMID: 23912822
Genetic analysis of TP53 in childhood myelodysplastic syndrome and juvenile myelomonocytic leukemia.
Saito S, Matsuda K, Taira C, Sano K, Tanaka-Yanagisawa M, Yanagisawa R, Nakazawa Y, Sakashita K, Shiohara M, Koike K
Leuk Res 2011 Dec;35(12):1578-84. Epub 2011 Jul 23 doi: 10.1016/j.leukres.2011.06.027. PMID: 21784522
Childhood myelodysplastic syndrome: focus on the approach to diagnosis and treatment of juvenile myelomonocytic leukemia.
Loh ML
Hematology Am Soc Hematol Educ Program 2010;2010:357-62. doi: 10.1182/asheducation-2010.1.357. PMID: 21239819
Childhood myelodysplastic syndrome in Denmark: incidence and predisposing conditions.
Hasle H, Kerndrup G, Jacobsen BB
Leukemia 1995 Sep;9(9):1569-72. PMID: 7658725

Therapy

Wilms' tumor 1 mRNA expression: a good tool for differentiating between myelodysplastic syndrome and aplastic anemia in children?
Zuo Y, Cheng Y, Zhang L, Qin Y, Luo H
Hematology 2019 Dec;24(1):480-486. doi: 10.1080/16078454.2019.1631507. PMID: 31210595
Comparison of long-term outcomes between children with aplastic anemia and refractory cytopenia of childhood who received immunosuppressive therapy with antithymocyte globulin and cyclosporine.
Hama A, Takahashi Y, Muramatsu H, Ito M, Narita A, Kosaka Y, Tsuchida M, Kobayashi R, Ito E, Yabe H, Ohga S, Ohara A, Kojima S
Haematologica 2015 Nov;100(11):1426-33. Epub 2015 Aug 13 doi: 10.3324/haematol.2015.128553. PMID: 26273061Free PMC Article
Bone marrow immunophenotyping by flow cytometry in refractory cytopenia of childhood.
Aalbers AM, van den Heuvel-Eibrink MM, Baumann I, Dworzak M, Hasle H, Locatelli F, De Moerloose B, Schmugge M, Mejstrikova E, Nováková M, Zecca M, Zwaan CM, Te Marvelde JG, Langerak AW, van Dongen JJ, Pieters R, Niemeyer CM, van der Velden VH
Haematologica 2015 Mar;100(3):315-23. Epub 2014 Nov 25 doi: 10.3324/haematol.2014.107706. PMID: 25425683Free PMC Article
Complex karyotype newly defined: the strongest prognostic factor in advanced childhood myelodysplastic syndrome.
Göhring G, Michalova K, Beverloo HB, Betts D, Harbott J, Haas OA, Kerndrup G, Sainati L, Bergstraesser E, Hasle H, Stary J, Trebo M, van den Heuvel-Eibrink MM, Zecca M, van Wering ER, Fischer A, Noellke P, Strahm B, Locatelli F, Niemeyer CM, Schlegelberger B
Blood 2010 Nov 11;116(19):3766-9. Epub 2010 Aug 27 doi: 10.1182/blood-2010-04-280313. PMID: 20802024
Intensive chemotherapy in childhood myelodysplastic syndrome. A comparison with results in acute myeloid leukemia.
Hasle H, Kerndrup G, Yssing M, Clausen N, Ostergaard E, Jacobsen N, Jacobsen BB
Leukemia 1996 Aug;10(8):1269-73. PMID: 8709630

Prognosis

Childhood myelodysplastic syndrome.
Chatterjee T, Choudhry VP
Indian J Pediatr 2013 Sep;80(9):764-71. Epub 2013 Aug 3 doi: 10.1007/s12098-013-1130-8. PMID: 23912822
Childhood myelodysplastic syndrome: focus on the approach to diagnosis and treatment of juvenile myelomonocytic leukemia.
Loh ML
Hematology Am Soc Hematol Educ Program 2010;2010:357-62. doi: 10.1182/asheducation-2010.1.357. PMID: 21239819
Comparison of childhood myelodysplastic syndrome, AML FAB M6 or M7, CCG 2891: report from the Children's Oncology Group.
Barnard DR, Alonzo TA, Gerbing RB, Lange B, Woods WG; Children's Oncology Group
Pediatr Blood Cancer 2007 Jul;49(1):17-22. doi: 10.1002/pbc.20951. PMID: 16856158
The International Prognostic Scoring System (IPSS) for childhood myelodysplastic syndrome (MDS) and juvenile myelomonocytic leukemia (JMML).
Hasle H, Baumann I, Bergsträsser E, Fenu S, Fischer A, Kardos G, Kerndrup G, Locatelli F, Rogge T, Schultz KR, Starý J, Trebo M, van den Heuvel-Eibrink MM, Harbott J, Nöllke P, Niemeyer CM; European Working Group on childhood MDS
Leukemia 2004 Dec;18(12):2008-14. doi: 10.1038/sj.leu.2403489. PMID: 15496981
Childhood myelodysplastic syndrome in Denmark: incidence and predisposing conditions.
Hasle H, Kerndrup G, Jacobsen BB
Leukemia 1995 Sep;9(9):1569-72. PMID: 7658725

Clinical prediction guides

Comparison of long-term outcomes between children with aplastic anemia and refractory cytopenia of childhood who received immunosuppressive therapy with antithymocyte globulin and cyclosporine.
Hama A, Takahashi Y, Muramatsu H, Ito M, Narita A, Kosaka Y, Tsuchida M, Kobayashi R, Ito E, Yabe H, Ohga S, Ohara A, Kojima S
Haematologica 2015 Nov;100(11):1426-33. Epub 2015 Aug 13 doi: 10.3324/haematol.2015.128553. PMID: 26273061Free PMC Article
Childhood myelodysplastic syndrome.
Chatterjee T, Choudhry VP
Indian J Pediatr 2013 Sep;80(9):764-71. Epub 2013 Aug 3 doi: 10.1007/s12098-013-1130-8. PMID: 23912822
Complex karyotype newly defined: the strongest prognostic factor in advanced childhood myelodysplastic syndrome.
Göhring G, Michalova K, Beverloo HB, Betts D, Harbott J, Haas OA, Kerndrup G, Sainati L, Bergstraesser E, Hasle H, Stary J, Trebo M, van den Heuvel-Eibrink MM, Zecca M, van Wering ER, Fischer A, Noellke P, Strahm B, Locatelli F, Niemeyer CM, Schlegelberger B
Blood 2010 Nov 11;116(19):3766-9. Epub 2010 Aug 27 doi: 10.1182/blood-2010-04-280313. PMID: 20802024
New recurrent deletions in the PPARgamma and TP53 genes are associated with childhood myelodysplastic syndrome.
Silveira CG, Oliveira FM, Valera ET, Ikoma MR, Borgonovo T, Cavalli IJ, Tone LG, Rogatto SR
Leuk Res 2009 Jan;33(1):19-27. Epub 2008 Sep 13 doi: 10.1016/j.leukres.2008.07.013. PMID: 18789822
The International Prognostic Scoring System (IPSS) for childhood myelodysplastic syndrome (MDS) and juvenile myelomonocytic leukemia (JMML).
Hasle H, Baumann I, Bergsträsser E, Fenu S, Fischer A, Kardos G, Kerndrup G, Locatelli F, Rogge T, Schultz KR, Starý J, Trebo M, van den Heuvel-Eibrink MM, Harbott J, Nöllke P, Niemeyer CM; European Working Group on childhood MDS
Leukemia 2004 Dec;18(12):2008-14. doi: 10.1038/sj.leu.2403489. PMID: 15496981

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