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Myeloproliferative neoplasm, unclassifiable

MedGen UID:
232365
•Concept ID:
C1333046
•
Neoplastic Process
Synonyms: chronic myeloproliferative disease, unclassifiable; Chronic myeloproliferative disease, unclassifiable; chronic myeloproliferative disorder, unclassifiable; CMPD, U; CMPD-U; MPN, U; MPN-U; Myeloproliferative neoplasm unclassifiable; myeloproliferative neoplasm, unclassifiable; unclassifiable chronic myeloproliferative disease; unclassifiable chronic myeloproliferative disorder; Undifferentiated myeloproliferative disease; undifferentiated myeloproliferative disease
SNOMED CT: Myeloproliferative neoplasm unclassifiable (1255668009)
 
Monarch Initiative: MONDO:0019452
Orphanet: ORPHA86830

Definition

Chronic myeloproliferative disease, unclassifiable is a hematological neoplasm characterized by clonal proliferation of myeloid precursors in the bone marrow, blood and other tissues (spleen, liver), with clinical, morphological and molecular features of myeloproliferative neoplasms (MPN), failing to meet criteria of a specific MPN. The presentation is nonspecific and variable and often includes leukocytosis, thrombocytosis and anemia. Splenomegaly, hepatomegaly as well as fatigue, malaise or weight loss may appear in advanced stages. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Myeloproliferative neoplasm, unclassifiable

Professional guidelines

PubMed

Diagnostic and management strategies for Myeloproliferative Neoplasm-Unclassifiable (MPN-U): An international survey of contemporary practice.
Hargreaves R, Harrison CN, McLornan DP
Curr Res Transl Med 2022 Jul;70(3):103338. Epub 2022 Feb 23 doi: 10.1016/j.retram.2022.103338. PMID: 35217310
Reclassification of subtypes in Philadelphia chromosome-negative myeloproliferative neoplasm by 2016 WHO diagnostic criteria: focus on the cases classified as myeloproliferative neoplasm, unclassifiable by the 2008 version.
Yun J, Kim JA, Park J, Im K, Lee YE, Jeong D, Ryu S, Lim KM, Kim SM, Ahn YO, Lee DS
Leuk Lymphoma 2020 Dec;61(14):3498-3502. Epub 2020 Sep 2 doi: 10.1080/10428194.2020.1808212. PMID: 32876501
Therapy-related myeloid neoplasms following treatment with radioiodine.
Schroeder T, Kuendgen A, Kayser S, Kröger N, Braulke F, Platzbecker U, Klärner V, Zohren F, Haase D, Stadler M, Schlenk R, Czibere AG, Bruns I, Fenk R, Gattermann N, Haas R, Kobbe G, Germing U
Haematologica 2012 Feb;97(2):206-12. Epub 2011 Oct 11 doi: 10.3324/haematol.2011.049114. PMID: 21993688Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Myeloproliferative Neoplasms, 2023

Recent clinical studies

Etiology

Advances in molecular evaluation of myeloproliferative neoplasms.
Li N, Chen M, Yin CC
Semin Diagn Pathol 2023 May;40(3):187-194. Epub 2023 Apr 13 doi: 10.1053/j.semdp.2023.04.007. PMID: 37087305
Diagnostic and management strategies for Myeloproliferative Neoplasm-Unclassifiable (MPN-U): An international survey of contemporary practice.
Hargreaves R, Harrison CN, McLornan DP
Curr Res Transl Med 2022 Jul;70(3):103338. Epub 2022 Feb 23 doi: 10.1016/j.retram.2022.103338. PMID: 35217310
How I manage myeloproliferative neoplasm-unclassifiable: Practical approaches for 2022 and beyond.
McLornan DP, Hargreaves R, Hernández-Boluda JC, Harrison CN
Br J Haematol 2022 May;197(4):407-416. Epub 2022 Feb 22 doi: 10.1111/bjh.18087. PMID: 35191542
Development of a myelodysplastic/myeloproliferative neoplasm-unclassifiable in a patient with acute myeloid leukemia: a case report and literature review.
Zhao M, Sun J, Liu S, Fan H, Fu Y, Tan Y, Gao S
J Int Med Res 2021 May;49(5):3000605211018426. doi: 10.1177/03000605211018426. PMID: 34057843Free PMC Article
The myeloproliferative neoplasms, unclassifiable: clinical and pathological considerations.
Gianelli U, Cattaneo D, Bossi A, Cortinovis I, Boiocchi L, Liu YC, Augello C, Bonometti A, Fiori S, Orofino N, Guidotti F, Orazi A, Iurlo A
Mod Pathol 2017 Feb;30(2):169-179. Epub 2016 Oct 14 doi: 10.1038/modpathol.2016.182. PMID: 27739437

Diagnosis

Advances in molecular evaluation of myeloproliferative neoplasms.
Li N, Chen M, Yin CC
Semin Diagn Pathol 2023 May;40(3):187-194. Epub 2023 Apr 13 doi: 10.1053/j.semdp.2023.04.007. PMID: 37087305
How I manage myeloproliferative neoplasm-unclassifiable: Practical approaches for 2022 and beyond.
McLornan DP, Hargreaves R, Hernández-Boluda JC, Harrison CN
Br J Haematol 2022 May;197(4):407-416. Epub 2022 Feb 22 doi: 10.1111/bjh.18087. PMID: 35191542
Myelodysplastic/myeloproliferative neoplasm, unclassifiable (MDS/MPN-U): More than just a "catch-all" term?
Shallis RM, Zeidan AM
Best Pract Res Clin Haematol 2020 Jun;33(2):101132. Epub 2019 Dec 6 doi: 10.1016/j.beha.2019.101132. PMID: 32460977
The myeloproliferative neoplasms, unclassifiable: clinical and pathological considerations.
Gianelli U, Cattaneo D, Bossi A, Cortinovis I, Boiocchi L, Liu YC, Augello C, Bonometti A, Fiori S, Orofino N, Guidotti F, Orazi A, Iurlo A
Mod Pathol 2017 Feb;30(2):169-179. Epub 2016 Oct 14 doi: 10.1038/modpathol.2016.182. PMID: 27739437
Molecular pathology of myeloproliferative neoplasms.
Klco JM, Vij R, Kreisel FH, Hassan A, Frater JL
Am J Clin Pathol 2010 Apr;133(4):602-15. doi: 10.1309/AJCPPPZ1WFVGNE4A. PMID: 20231614

Therapy

Acute myeloid leukemia developed through myeloproliferative features during immunosuppressive therapy for juvenile idiopathic arthritis.
Oura M, Sumitani R, Maeda Y, Yagi H, Takahashi M, Harada T, Fujii S, Miki H, Hori T, Murai J, Kagawa K, Abe M, Nakamura S
J Med Invest 2024;71(3.4):335-339. doi: 10.2152/jmi.71.335. PMID: 39462577
Prognostic Value of Pretreatment Fetal Hemoglobin Levels in Patients with Myelodysplastic Syndromes and Acute Myeloid Leukemia Treated with Azacitidine: A Single-center Retrospective Study.
Saga T, Kanagawa M, Harada T, Lang L, Yamawaki F, Ishihara T
Intern Med 2024 Mar 15;63(6):781-790. Epub 2023 Jul 26 doi: 10.2169/internalmedicine.1216-22. PMID: 37495538Free PMC Article
Successful prevention of severe allergic transfusion reactions with omalizumab.
Shi S, Han J, Yao Y, Xu Y, Wu D, Wang Y
Transfusion 2020 Jul;60(7):1639-1642. Epub 2020 Mar 5 doi: 10.1111/trf.15735. PMID: 32134497
Therapy-related myeloid neoplasms following treatment with radioiodine.
Schroeder T, Kuendgen A, Kayser S, Kröger N, Braulke F, Platzbecker U, Klärner V, Zohren F, Haase D, Stadler M, Schlenk R, Czibere AG, Bruns I, Fenk R, Gattermann N, Haas R, Kobbe G, Germing U
Haematologica 2012 Feb;97(2):206-12. Epub 2011 Oct 11 doi: 10.3324/haematol.2011.049114. PMID: 21993688Free PMC Article

Prognosis

Predictors of clinical outcome in myeloproliferative neoplasm, unclassifiable: A Bone Marrow Pathology Group study.
Crane GM, Geyer JT, Thakral B, Wang SA, Wool GD, Li KD, Davis AR, Boiocchi L, Bosler D, Bueso-Ramos CE, Arber DA, George TI, Bagg A, Hasserjian RP, Orazi A, Hsi ED, Rogers HJ
Am J Clin Pathol 2024 Sep 3;162(3):233-242. doi: 10.1093/ajcp/aqae033. PMID: 38597584
Diagnostic and management strategies for Myeloproliferative Neoplasm-Unclassifiable (MPN-U): An international survey of contemporary practice.
Hargreaves R, Harrison CN, McLornan DP
Curr Res Transl Med 2022 Jul;70(3):103338. Epub 2022 Feb 23 doi: 10.1016/j.retram.2022.103338. PMID: 35217310
How I manage myeloproliferative neoplasm-unclassifiable: Practical approaches for 2022 and beyond.
McLornan DP, Hargreaves R, Hernández-Boluda JC, Harrison CN
Br J Haematol 2022 May;197(4):407-416. Epub 2022 Feb 22 doi: 10.1111/bjh.18087. PMID: 35191542
Clinicopathological characterisation of myeloproliferative neoplasm-unclassifiable (MPN-U): a retrospective analysis from a large UK tertiary referral centre.
Deschamps P, Moonim M, Radia D, Curto-Garcia N, Woodley C, Bassiony S, O'Sullivan J, Harrington P, Raj K, Francis Y, Kordasti S, Ali S, Harrison CN, McLornan DP
Br J Haematol 2021 May;193(4):792-797. Epub 2021 Mar 9 doi: 10.1111/bjh.17375. PMID: 33751548
CALR, JAK2, and MPL mutation profiles in patients with four different subtypes of myeloproliferative neoplasms: primary myelofibrosis, essential thrombocythemia, polycythemia vera, and myeloproliferative neoplasm, unclassifiable.
Kim SY, Im K, Park SN, Kwon J, Kim JA, Lee DS
Am J Clin Pathol 2015 May;143(5):635-44. doi: 10.1309/AJCPUAAC16LIWZMM. PMID: 25873496

Clinical prediction guides

Predictors of clinical outcome in myeloproliferative neoplasm, unclassifiable: A Bone Marrow Pathology Group study.
Crane GM, Geyer JT, Thakral B, Wang SA, Wool GD, Li KD, Davis AR, Boiocchi L, Bosler D, Bueso-Ramos CE, Arber DA, George TI, Bagg A, Hasserjian RP, Orazi A, Hsi ED, Rogers HJ
Am J Clin Pathol 2024 Sep 3;162(3):233-242. doi: 10.1093/ajcp/aqae033. PMID: 38597584
Clinicopathological characterisation of myeloproliferative neoplasm-unclassifiable (MPN-U): a retrospective analysis from a large UK tertiary referral centre.
Deschamps P, Moonim M, Radia D, Curto-Garcia N, Woodley C, Bassiony S, O'Sullivan J, Harrington P, Raj K, Francis Y, Kordasti S, Ali S, Harrison CN, McLornan DP
Br J Haematol 2021 May;193(4):792-797. Epub 2021 Mar 9 doi: 10.1111/bjh.17375. PMID: 33751548
CALR, JAK2, and MPL mutation profiles in patients with four different subtypes of myeloproliferative neoplasms: primary myelofibrosis, essential thrombocythemia, polycythemia vera, and myeloproliferative neoplasm, unclassifiable.
Kim SY, Im K, Park SN, Kwon J, Kim JA, Lee DS
Am J Clin Pathol 2015 May;143(5):635-44. doi: 10.1309/AJCPUAAC16LIWZMM. PMID: 25873496
JAK2-v617F mutation is associated with clinical and laboratory features of myeloproliferative neoplasms.
Duletić AN, Dekanić A, Hadzisejdić I, Kusen I, Matusan-Ilijas K, Grohovac D, Grahovac B, Jonjić N
Coll Antropol 2012 Sep;36(3):859-65. PMID: 23213945
Therapy-related myeloid neoplasms following treatment with radioiodine.
Schroeder T, Kuendgen A, Kayser S, Kröger N, Braulke F, Platzbecker U, Klärner V, Zohren F, Haase D, Stadler M, Schlenk R, Czibere AG, Bruns I, Fenk R, Gattermann N, Haas R, Kobbe G, Germing U
Haematologica 2012 Feb;97(2):206-12. Epub 2011 Oct 11 doi: 10.3324/haematol.2011.049114. PMID: 21993688Free PMC Article

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    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Myeloproliferative Neoplasms, 2023

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