Clinicopathological characterisation of myeloproliferative neoplasm-unclassifiable (MPN-U): a retrospective analysis from a large UK tertiary referral centre

被引：12

作者：

Deschamps, Paul ^{[1
,2
]}

Moonim, Mufaddal ^{[1
,3
]}

Radia, Deepti ^{[1
]}

Curto-Garcia, Natalia ^{[1
]}

Woodley, Claire ^{[1
]}

Bassiony, Sarah ^{[1
]}

O'Sullivan, Jennifer ^{[1
]}

Harrington, Patrick ^{[1
]}

Raj, Kavita ^{[1
]}

Francis, Yvonne ^{[1
]}

Kordasti, Shahram ^{[1
,4
]}

Ali, Sahra ^{[1
]}

Harrison, Claire N. ^{[1
]}

McLornan, Donal P. ^{[1
]}

机构：

[1] Guys & St Thomas NHS Fdn Trust, Dept Haematol, London, England

[2] Hop Cochin, AP HP, Serv Hematol, Paris, France

[3] Guys & St Thomas NHS Fdn Trust, Histopathol Dept, London, England

[4] Kings Coll London, Comprehens Canc Ctr, Syst Canc Immunol, London, England

来源：

BRITISH JOURNAL OF HAEMATOLOGY | 2021年 / 193卷 / 04期

关键词：

clinical data; genomics; myeloproliferative neoplasms‐ unclassifiable; outcome; STEM-CELL TRANSPLANTATION; WORKING PARTY; SURVIVAL; LEUKEMIA; CRITERIA; IMPACT;

D O I：

10.1111/bjh.17375

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Myeloproliferative neoplasm-unclassifiable (MPN-U) presents an MPN-type phenotype that fails to meet diagnostic criteria for other MPN variants. Variability in the clinicopathological phenotypes presents many challenges. Amongst a registry cohort of 1512 patients with MPN, 82 with MPN-U were included, with a median (range) age of 49 center dot 7 (13-79) years. Albeit heterogeneous, common presentation features included raised lactate dehydrogenase, thrombocytosis and clustered/pleomorphic megakaryocytes on trephine biopsy. Thrombosis was common (21%), necessitating vigilance. The median event-free survival was 11 center dot 25 years (95% confidence interval 9 center dot 3-not reached), significantly shortened in cases with lower platelet counts (<500 x 10(9)/l) and a leucocytosis (>= 12 x 10(9)/l) at presentation. Generation of potential MPN-U prognostic scores is required.

引用

页码：792 / 797

页数：6

共 12 条

[1] The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia [J].

Arber, Daniel A. ;

Orazi, Attilio ;

Hasserjian, Robert ;

Thiele, Jurgen ;

Borowitz, Michael J. ;

Le Beau, Michelle M. ;

Bloomfield, Clara D. ;

Cazzola, Mario ;

Vardiman, James W. .

BLOOD, 2016, 127 (20) :2391-2405

[2] Diagnostic impact of the 2016 revised who criteria for polycythemia vera [J].

Barbui, Tiziano ;

Thiele, Juergen ;

Gisslinger, Heinz ;

Carobbio, Alessandra ;

Vannucchi, Alessandro M. ;

Tefferi, Ayalew .

AMERICAN JOURNAL OF HEMATOLOGY, 2017, 92 (05) :417-419

[3] Myeloproliferative neoplasms: Morphology and clinical practice [J].

Barbui, Tiziano ;

Thiele, Juergen ;

Vannucchi, Alessandro M. ;

Tefferi, Ayalew .

AMERICAN JOURNAL OF HEMATOLOGY, 2016, 91 (04) :430-433

[4] The myeloproliferative neoplasms, unclassifiable: clinical and pathological considerations [J].

Gianelli, Umberto ;

Cattaneo, Daniele ;

Bossi, Anna ;

Cortinovis, Ivan ;

Boiocchi, Leonardo ;

Liu, Yen-Chun ;

Augello, Claudia ;

Bonometti, Arturo ;

Fiori, Stefano ;

Orofino, Nicola ;

Guidotti, Francesca ;

Orazi, Attilio ;

Iurlo, Alessandra .

MODERN PATHOLOGY, 2017, 30 (02) :169-179

[5] Patterns of Survival Among Patients With Myeloproliferative Neoplasms Diagnosed in Sweden From 1973 to 2008: A Population-Based Study [J].

Hultcrantz, Malin ;

Kristinsson, Sigurdur Yngvi ;

Andersson, Therese M. -L. ;

Landgren, Ola ;

Eloranta, Sandra ;

Derolf, Asa Rangert ;

Dickman, Paul W. ;

Bjorkholm, Magnus .

JOURNAL OF CLINICAL ONCOLOGY, 2012, 30 (24) :2995-3001

[6] Impact of the 2016 revised WHO criteria for myeloproliferative neoplasms, unclassifiable: Comparison with the 2008 version [J].

Iurlo, Alessandra ;

Gianelli, Umberto ;

Cattaneo, Daniele ;

Thiele, Juergen ;

Orazi, Attilio .

AMERICAN JOURNAL OF HEMATOLOGY, 2017, 92 (04) :E48-E51

[7] Higher busulfan dose intensity appears to improve leukemia-free and overall survival in AML allografted in CR2: An analysis from the Acute Leukemia Working Party of the European Group for Blood and Marrow Transplantation [J].

Kharfan-Dabaja, Mohamed A. ;

Labopin, Myriam ;

Bazarbachi, Ali ;

Socie, Gerard ;

Kroeger, Nicolaus ;

Blaise, Didier ;

Veelken, Hendrik ;

Bermudez, Arancha ;

Or, Reuven ;

Lioure, Bruno ;

Beelen, Dietrich ;

Fegueux, Nathalie ;

Hamladji, Rose Marie ;

Nagler, Arnon ;

Mohty, Mohamad .

LEUKEMIA RESEARCH, 2015, 39 (09) :933-937

[8] Outcome of Allogeneic Haematopoietic Stem Cell Transplantation in Myeloproliferative NeoplasmsUnclassifiable: A Retrospective Study By the Chronic Malignancies Working Party of EBMT [J].

McLornan, Donal P. ;

Malpassuti, Vittoria ;

Iacobelli, Simona ;

Lippinkhof-Kozijn, Anne ;

Koster, Linda ;

Robin, Marie ;

Chalandon, Yves ;

Bunjes, Donald ;

Beelen, Dietrich W. ;

Potter, Victoria ;

Sengeloev, Henrik ;

Radujkovic, Aleksandar ;

Passweg, Jakob R. ;

Kroeger, Nicolaus ;

Wulf, Gerald ;

Johansson, Jan Erik ;

Ciceri, Fabio ;

Bornhaeuser, Martin ;

Holler, Ernst ;

Beguin, Yves ;

Lioure, Bruno ;

Martin, Sonja ;

Milojkovic, Dragana ;

Hayden, Patrick ;

Hernandez Boluda, Juan Carlos ;

Czerw, Tomasz ;

yakoub-Agha, Ibrahim .

BLOOD, 2019, 134

[9]

Medistica, PVALUEIO GUI R STAT

[10] Myeloproliferative and lymphoproliferative disorders: State of the art [J].

Rumi, Elisa ;

Barate, Claudia ;

Benevolo, Giulia ;

Maffioli, Margherita ;

Ricco, Alessandra ;

Sant'Antonio, Emanuela .

HEMATOLOGICAL ONCOLOGY, 2020, 38 (02) :121-128

← 1 2 →