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Unclassified myelodysplastic/myeloproliferative disease

MedGen UID:
272202
Concept ID:
C1328061
Neoplastic Process
Synonyms: Mixed Myelodysplastic/Myeloproliferative Disease, Unclassifiable; Mixed Myeloproliferative/Myelodysplastic Syndrome, Unclassifiable; Myelodysplastic/Myeloproliferative Disease, Unclassifiable; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Myelodysplastic/myeloproliferative neoplasm, unclassifiable; Myeloproliferative neoplasm, unclassifiable; Unclassifiable Myelodysplastic/Myeloproliferative Disease; Unclassifiable Myeloproliferative/Myelodysplastic Syndrome; Unclassified mixed myelodysplastic/myeloproliferatic syndrome
SNOMED CT: Myelodysplastic/myeloproliferative neoplasm, unclassifiable (447240002); Myelodysplastic/myeloproliferative neoplasm, unclassifiable (447596005); Myeloproliferative neoplasm, unclassifiable (447240002)
 
Orphanet: ORPHA98825

Definition

A rare myelodysplastic/myeloproliferative neoplasm characterized by clinical, laboratory, and morphological features of both myelodysplastic syndrome and myeloproliferative neoplasm at onset, in the absence of recent cytotoxic or growth factor therapy, and without Philadelphia chromosome, BCR-ABL1 or PCM1-JAK2 fusion, or rearrangement of PDGFRA, PDGFRB, or FGFR1. Cases of a previously well-defined myeloproliferative neoplasm developing dysplastic features are excluded, and the criteria for any other myelodysplastic/myeloproliferative neoplasm, myelodysplastic syndrome, or myeloproliferative neoplasm are not met. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUnclassified myelodysplastic/myeloproliferative disease

Professional guidelines

PubMed

DiNardo CD, Daver N, Jain N, Pemmaraju N, Bueso-Ramos C, Yin CC, Pierce S, Jabbour E, Cortes JE, Kantarjian HM, Garcia-Manero G, Verstovsek S
Leukemia 2014 Apr;28(4):958-61. Epub 2014 Jan 10 doi: 10.1038/leu.2014.8. PMID: 24492324Free PMC Article

Recent clinical studies

Etiology

Hunter AM, Padron E
Best Pract Res Clin Haematol 2020 Sep;33(3):101195. Epub 2020 Jul 5 doi: 10.1016/j.beha.2020.101195. PMID: 33038984
Zhang H, Wilmot B, Bottomly D, Dao KT, Stevens E, Eide CA, Khanna V, Rofelty A, Savage S, Reister Schultz A, Long N, White L, Carlos A, Henson R, Lin C, Searles R, Collins RH, DeAngelo DJ, Deininger MW, Dunn T, Hein T, Luskin MR, Medeiros BC, Oh ST, Pollyea DA, Steensma DP, Stone RM, Druker BJ, McWeeney SK, Maxson JE, Gotlib JR, Tyner JW
Blood 2019 Sep 12;134(11):867-879. Epub 2019 Jul 31 doi: 10.1182/blood.2019000611. PMID: 31366621Free PMC Article
Smith BN, Savona M, Komrokji RS
Clin Lymphoma Myeloma Leuk 2019 Jan;19(1):1-8. Epub 2018 Nov 23 doi: 10.1016/j.clml.2018.11.019. PMID: 30555034Free PMC Article
Hahm C, Huh HJ, Mun YC, Seong CM, Chung WS, Huh J
Int J Lab Hematol 2015 Apr;37(2):181-9. Epub 2014 May 21 doi: 10.1111/ijlh.12257. PMID: 24845343
Orazi A, Germing U
Leukemia 2008 Jul;22(7):1308-19. Epub 2008 May 15 doi: 10.1038/leu.2008.119. PMID: 18480833

Diagnosis

Patnaik MM, Lasho TL
Hematology Am Soc Hematol Educ Program 2020 Dec 4;2020(1):450-459. doi: 10.1182/hematology.2020000130. PMID: 33275756Free PMC Article
Palomo L, Meggendorfer M, Hutter S, Twardziok S, Ademà V, Fuhrmann I, Fuster-Tormo F, Xicoy B, Zamora L, Acha P, Kerr CM, Kern W, Maciejewski JP, Solé F, Haferlach C, Haferlach T
Blood 2020 Oct 15;136(16):1851-1862. doi: 10.1182/blood.2019004229. PMID: 32573691Free PMC Article
Zhang H, Wilmot B, Bottomly D, Dao KT, Stevens E, Eide CA, Khanna V, Rofelty A, Savage S, Reister Schultz A, Long N, White L, Carlos A, Henson R, Lin C, Searles R, Collins RH, DeAngelo DJ, Deininger MW, Dunn T, Hein T, Luskin MR, Medeiros BC, Oh ST, Pollyea DA, Steensma DP, Stone RM, Druker BJ, McWeeney SK, Maxson JE, Gotlib JR, Tyner JW
Blood 2019 Sep 12;134(11):867-879. Epub 2019 Jul 31 doi: 10.1182/blood.2019000611. PMID: 31366621Free PMC Article
Smith BN, Savona M, Komrokji RS
Clin Lymphoma Myeloma Leuk 2019 Jan;19(1):1-8. Epub 2018 Nov 23 doi: 10.1016/j.clml.2018.11.019. PMID: 30555034Free PMC Article
Cazzola M, Malcovati L, Invernizzi R
Hematology Am Soc Hematol Educ Program 2011;2011:264-72. doi: 10.1182/asheducation-2011.1.264. PMID: 22160044

Therapy

Patnaik MM, Lasho T
Hematology Am Soc Hematol Educ Program 2020 Dec 4;2020(1):460-464. doi: 10.1182/hematology.2020000163. PMID: 33275673Free PMC Article
Hu Z, Hu S, Ji C, Tang Z, Thakral B, Loghavi S, Medeiros LJ, Wang W
Leuk Res 2018 Feb;65:25-28. Epub 2017 Dec 23 doi: 10.1016/j.leukres.2017.12.004. PMID: 29288910
Mekinian A, Grignano E, Braun T, Decaux O, Liozon E, Costedoat-Chalumeau N, Kahn JE, Hamidou M, Park S, Puéchal X, Toussirot E, Falgarone G, Launay D, Morel N, Trouiller S, Mathian A, Gombert B, Schoindre Y, Lioger B, De Wazieres B, Amoura Z, Buchdaul AL, Georgin-Lavialle S, Dion J, Madaule S, Raffray L, Cathebras P, Piette JC, Rose C, Ziza JM, Lortholary O, Montestruc F, Omouri M, Denis G, Rossignol J, Nimubona S, Adès L, Gardin C, Fenaux P, Fain O
Rheumatology (Oxford) 2016 Feb;55(2):291-300. Epub 2015 Sep 8 doi: 10.1093/rheumatology/kev294. PMID: 26350487
Gianelli U, Iurlo A, Cattaneo D, Lambertenghi-Deliliers G
Expert Rev Hematol 2014 Apr;7(2):255-64. Epub 2014 Feb 13 doi: 10.1586/17474086.2014.876898. PMID: 24524231
Bejanyan N, Tiu RV, Raza A, Jankowska A, Kalaycio M, Advani A, Chan J, Saunthararajah Y, Mooney L, Maciejewski JP, Sekeres MA
Cancer 2012 Aug 15;118(16):3968-76. Epub 2011 Dec 16 doi: 10.1002/cncr.26741. PMID: 22180010

Prognosis

Patnaik MM, Lasho TL
Hematology Am Soc Hematol Educ Program 2020 Dec 4;2020(1):450-459. doi: 10.1182/hematology.2020000130. PMID: 33275756Free PMC Article
Patnaik MM, Lasho T
Hematology Am Soc Hematol Educ Program 2020 Dec 4;2020(1):460-464. doi: 10.1182/hematology.2020000163. PMID: 33275673Free PMC Article
Palomo L, Meggendorfer M, Hutter S, Twardziok S, Ademà V, Fuhrmann I, Fuster-Tormo F, Xicoy B, Zamora L, Acha P, Kerr CM, Kern W, Maciejewski JP, Solé F, Haferlach C, Haferlach T
Blood 2020 Oct 15;136(16):1851-1862. doi: 10.1182/blood.2019004229. PMID: 32573691Free PMC Article
Zhang H, Wilmot B, Bottomly D, Dao KT, Stevens E, Eide CA, Khanna V, Rofelty A, Savage S, Reister Schultz A, Long N, White L, Carlos A, Henson R, Lin C, Searles R, Collins RH, DeAngelo DJ, Deininger MW, Dunn T, Hein T, Luskin MR, Medeiros BC, Oh ST, Pollyea DA, Steensma DP, Stone RM, Druker BJ, McWeeney SK, Maxson JE, Gotlib JR, Tyner JW
Blood 2019 Sep 12;134(11):867-879. Epub 2019 Jul 31 doi: 10.1182/blood.2019000611. PMID: 31366621Free PMC Article
Cazzola M, Malcovati L, Invernizzi R
Hematology Am Soc Hematol Educ Program 2011;2011:264-72. doi: 10.1182/asheducation-2011.1.264. PMID: 22160044

Clinical prediction guides

Lee MY, Jang S, Park CJ, Cho YU
Clin Lab 2022 Apr 1;68(4) doi: 10.7754/Clin.Lab.2021.210738. PMID: 35443589
Kanagal-Shamanna R, Orazi A, Hasserjian RP, Arber DA, Reichard K, Hsi ED, Bagg A, Rogers HJ, Geyer J, Darbaniyan F, Do KA, Devins KM, Pozdnyakova O, George TI, Cin PD, Greipp PT, Routbort MJ, Patel K, Garcia-Manero G, Verstovsek S, Medeiros LJ, Wang SA, Bueso-Ramos C
Mod Pathol 2022 Apr;35(4):470-479. Epub 2021 Nov 13 doi: 10.1038/s41379-021-00961-0. PMID: 34775472Free PMC Article
Calvo X, Garcia-Gisbert N, Parraga I, Gibert J, Florensa L, Andrade-Campos M, Merchan B, Garcia-Avila S, Montesdeoca S, Fernández-Rodríguez C, Salido M, Puiggros A, Espinet B, Colomo L, Roman-Bravo D, Bellosillo B, Ferrer A, Arenillas L
Blood Adv 2020 Oct 27;4(20):5285-5296. doi: 10.1182/bloodadvances.2020002206. PMID: 33108455Free PMC Article
Palomo L, Meggendorfer M, Hutter S, Twardziok S, Ademà V, Fuhrmann I, Fuster-Tormo F, Xicoy B, Zamora L, Acha P, Kerr CM, Kern W, Maciejewski JP, Solé F, Haferlach C, Haferlach T
Blood 2020 Oct 15;136(16):1851-1862. doi: 10.1182/blood.2019004229. PMID: 32573691Free PMC Article
Zhang H, Wilmot B, Bottomly D, Dao KT, Stevens E, Eide CA, Khanna V, Rofelty A, Savage S, Reister Schultz A, Long N, White L, Carlos A, Henson R, Lin C, Searles R, Collins RH, DeAngelo DJ, Deininger MW, Dunn T, Hein T, Luskin MR, Medeiros BC, Oh ST, Pollyea DA, Steensma DP, Stone RM, Druker BJ, McWeeney SK, Maxson JE, Gotlib JR, Tyner JW
Blood 2019 Sep 12;134(11):867-879. Epub 2019 Jul 31 doi: 10.1182/blood.2019000611. PMID: 31366621Free PMC Article

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