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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

Loh ML
Hematology Am Soc Hematol Educ Program 2010;2010:357-62. doi: 10.1182/asheducation-2010.1.357. PMID: 21239819
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

Yoo JW, Im HJ, Kim H, Koh KN, Kang SH, Min SY, Choi ES, Jang S, Park CJ, Seo JJ
Bone Marrow Transplant 2020 Aug;55(8):1595-1603. Epub 2020 Feb 13 doi: 10.1038/s41409-020-0814-8. PMID: 32054998
Iwafuchi H, Ito M
Histopathology 2019 Jan;74(2):239-247. Epub 2018 Oct 29 doi: 10.1111/his.13721. PMID: 30062702
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
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
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

Diagnosis

Chisholm KM, Bohling SD
Clin Lab Med 2023 Dec;43(4):639-655. Epub 2023 Aug 7 doi: 10.1016/j.cll.2023.06.005. PMID: 37865508
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
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
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
Loh ML
Hematology Am Soc Hematol Educ Program 2010;2010:357-62. doi: 10.1182/asheducation-2010.1.357. PMID: 21239819

Therapy

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
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
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
Sevilla J, Fernández-Plaza S, Lassaletta A, González-Vicent M, Contra T, Madero L
J Pediatr Hematol Oncol 2007 Dec;29(12):839-40. doi: 10.1097/MPH.0b013e31815814c9. PMID: 18090933
Hasle H, Kerndrup G, Yssing M, Clausen N, Ostergaard E, Jacobsen N, Jacobsen BB
Leukemia 1996 Aug;10(8):1269-73. PMID: 8709630

Prognosis

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
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
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
Hasle H, Wadsworth LD, Massing BG, McBride M, Schultz KR
Br J Haematol 1999 Sep;106(4):1027-32. doi: 10.1046/j.1365-2141.1999.01645.x. PMID: 10520007
Hasle H, Kerndrup G, Jacobsen BB
Leukemia 1995 Sep;9(9):1569-72. PMID: 7658725

Clinical prediction guides

Yoo JW, Im HJ, Kim H, Koh KN, Kang SH, Min SY, Choi ES, Jang S, Park CJ, Seo JJ
Bone Marrow Transplant 2020 Aug;55(8):1595-1603. Epub 2020 Feb 13 doi: 10.1038/s41409-020-0814-8. PMID: 32054998
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
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
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
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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