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Unclassified myelodysplastic/myeloproliferative disease

MedGen UID:
272202
Concept ID:
C1328061
Neoplastic Process
Synonyms: Chronic Myeloproliferative Disease, Unclassifiable; Chronic Myeloproliferative Disorder, Unclassifiable; Myelodysplastic/myeloproliferative neoplasm, unclassifiable; Myeloproliferative Neoplasm, NOS; Myeloproliferative Neoplasm, Not Otherwise Specified; Myeloproliferative neoplasm, unclassifiable; Myeloproliferative Neoplasm, Unclassifiable; Unclassifiable Chronic Myeloproliferative Disease; Unclassifiable Chronic Myeloproliferative Disorder; 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

Molony P, Smith AC, Selvarajah S, Sakhdari A
Cytogenet Genome Res 2021;161(12):564-568. Epub 2022 Jan 17 doi: 10.1159/000521509. PMID: 35038703Free PMC Article
Mangaonkar AA, Swoboda DM, Lasho TL, Finke C, Ketterling RP, Reichard KK, Padron E, Talati C, Patnaik MM
Leukemia 2021 Nov;35(11):3329-3333. Epub 2021 Apr 30 doi: 10.1038/s41375-021-01258-6. PMID: 33931725
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
Macedo LC, de Cesare Quintero F, Pagliari-E-Silva S, Pagnano KB, Rodrigues C, de Alencar JB, Sell AM, Visentainer JE
Blood Cells Mol Dis 2016 Mar;57:54-7. Epub 2015 Dec 10 doi: 10.1016/j.bcmd.2015.12.005. PMID: 26852656

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
Macedo LC, de Cesare Quintero F, Pagliari-E-Silva S, Pagnano KB, Rodrigues C, de Alencar JB, Sell AM, Visentainer JE
Blood Cells Mol Dis 2016 Mar;57:54-7. Epub 2015 Dec 10 doi: 10.1016/j.bcmd.2015.12.005. PMID: 26852656
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

Wang Z, Wan L, Lin D, Li CW, Tian Z, Mi YC
Acta Haematol 2022;145(5):560-565. Epub 2022 Mar 25 doi: 10.1159/000524275. PMID: 35340014Free 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
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

Prognosis

Mangaonkar AA, Swoboda DM, Lasho TL, Finke C, Ketterling RP, Reichard KK, Padron E, Talati C, Patnaik MM
Leukemia 2021 Nov;35(11):3329-3333. Epub 2021 Apr 30 doi: 10.1038/s41375-021-01258-6. PMID: 33931725
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
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
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

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
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
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

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