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000275763 0247_ $$2doi$$a10.1182/bloodadvances.2022008292
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000275763 041__ $$aEnglish
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000275763 1001_ $$00000-0002-1912-4076$$aKennedy, Vanessa E$$b0
000275763 245__ $$aMast cell leukemia: clinical and molecular features and survival outcomes of patients in the ECNM Registry.
000275763 260__ $$aWashington, DC$$bAmerican Society of Hematology$$c2023
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000275763 520__ $$aMast cell leukemia (MCL) is a rare subtype of systemic mastocytosis defined by ≥20% mast cells (MC) on a bone marrow aspirate. We evaluated 92 patients with MCL from the European Competence Network on Mastocytosis registry. Thirty-one (34%) patients had a diagnosis of MCL with an associated hematologic neoplasm (MCL-AHN). Chronic MCL (lack of C-findings) comprised 14% of patients, and only 4.5% had 'leukemic MCL' (≥10% circulating MCs). KIT D816V was found in 62/85 (73%) evaluable patients; 9 (11%) individuals exhibited alternative KIT mutations, and no KIT variants were detected in 14 (17%) subjects. Ten evaluable patients (17%) had an abnormal karyotype and the poor-risk SRSF2, ASXL1, and RUNX1 (S/A/R) mutations were identified in 16/36 (44%) patients who underwent next-generation sequencing. Midostaurin was the most common therapy administered to 65% of patients and 45% as first-line therapy. The median overall survival (OS) was 1.6 years. In multivariate analysis (S/A/R mutations excluded owing to low event rates), a diagnosis of MCL-AHN (hazard ratio [HR], 4.7; 95% confidence interval [CI], 1.7-13.0; P = .001) and abnormal karyotype (HR, 5.6; 95% CI, 1.4-13.3; P = .02) were associated with inferior OS; KIT D816V positivity (HR, 0.33; 95% CI, 0.11-0.98; P = .04) and midostaurin treatment (HR, 0.32; 95% CI, 0.08-0.72; P = .008) were associated with superior OS. These data provide the most comprehensive snapshot of the clinicopathologic, molecular, and treatment landscape of MCL to date, and should help further inform subtyping and prognostication of MCL.
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000275763 650_2 $$2MeSH$$aHumans
000275763 650_2 $$2MeSH$$aLeukemia, Mast-Cell: diagnosis
000275763 650_2 $$2MeSH$$aLeukemia, Mast-Cell: genetics
000275763 650_2 $$2MeSH$$aMastocytosis, Systemic: diagnosis
000275763 650_2 $$2MeSH$$aMastocytosis, Systemic: drug therapy
000275763 650_2 $$2MeSH$$aMastocytosis, Systemic: genetics
000275763 650_2 $$2MeSH$$aMastocytosis
000275763 650_2 $$2MeSH$$aMast Cells
000275763 650_2 $$2MeSH$$aAbnormal Karyotype
000275763 7001_ $$aPerkins, Cecelia$$b1
000275763 7001_ $$aReiter, Andreas$$b2
000275763 7001_ $$00000-0001-9148-9398$$aJawhar, Mohamad$$b3
000275763 7001_ $$00000-0003-0767-1504$$aLübke, Johannes$$b4
000275763 7001_ $$00000-0003-2617-9427$$aKluin-Nelemans, Hanneke C$$b5
000275763 7001_ $$00000-0001-6500-350X$$aShomali, William$$b6
000275763 7001_ $$aLangford, Cheryl$$b7
000275763 7001_ $$aAbuel, Justin$$b8
000275763 7001_ $$aHermine, Olivier$$b9
000275763 7001_ $$aNiedoszytko, Marek$$b10
000275763 7001_ $$aGorska, Aleksandra$$b11
000275763 7001_ $$aMital, Andrzej$$b12
000275763 7001_ $$aBonadonna, Patrizia$$b13
000275763 7001_ $$aZanotti, Roberta$$b14
000275763 7001_ $$00000-0001-8177-374X$$aTanasi, Ilaria$$b15
000275763 7001_ $$aMattsson, Mattias$$b16
000275763 7001_ $$aHagglund, Hans$$b17
000275763 7001_ $$aTriggiani, Massimo$$b18
000275763 7001_ $$aYavuz, Akif Selim$$b19
000275763 7001_ $$00000-0001-6316-3112$$aPanse, Jens$$b20
000275763 7001_ $$00000-0003-2839-5983$$aChristen, Deborah$$b21
000275763 7001_ $$00000-0003-3818-8638$$aHeizmann, Marc$$b22
000275763 7001_ $$0P:(DE-HGF)0$$aShoumariyeh, Khalid$$b23
000275763 7001_ $$aMüller, Sabine$$b24
000275763 7001_ $$00000-0002-0058-7142$$aElena, Chiara$$b25
000275763 7001_ $$00000-0002-1460-1611$$aMalcovati, Luca$$b26
000275763 7001_ $$aFiorelli, Nicolas$$b27
000275763 7001_ $$aWortmann, Friederike$$b28
000275763 7001_ $$aVucinic, Vladan$$b29
000275763 7001_ $$00000-0002-2775-3681$$aBrockow, Knut$$b30
000275763 7001_ $$00000-0002-5041-3167$$aFokoloros, Christos$$b31
000275763 7001_ $$00000-0002-3376-837X$$aPapageorgiou, Sotirios G$$b32
000275763 7001_ $$00000-0002-7368-1068$$aBreynaert, Christine$$b33
000275763 7001_ $$00000-0002-4595-9579$$aBullens, Dominique$$b34
000275763 7001_ $$aDoubek, Michael$$b35
000275763 7001_ $$aIlerhaus, Anja$$b36
000275763 7001_ $$aAngelova-Fischer, Irena$$b37
000275763 7001_ $$aSolomianyi, Oleksii$$b38
000275763 7001_ $$aVárkonyi, Judit$$b39
000275763 7001_ $$aSabato, Vito$$b40
000275763 7001_ $$aRüfer, Axel$$b41
000275763 7001_ $$aSchug, Tanja Daniela$$b42
000275763 7001_ $$aHermans, Maud A W$$b43
000275763 7001_ $$00000-0001-5791-0775$$aFortina, Anna Belloni$$b44
000275763 7001_ $$00000-0003-3583-0816$$aCaroppo, Francesca$$b45
000275763 7001_ $$00000-0002-3706-3166$$aBumbea, Horia$$b46
000275763 7001_ $$00000-0002-1683-8882$$aGulen, Theo$$b47
000275763 7001_ $$00000-0002-4595-8226$$aHartmann, Karin$$b48
000275763 7001_ $$00000-0003-2844-2196$$aElberink, Hanneke Oude$$b49
000275763 7001_ $$00000-0001-9208-5264$$aSchwaab, Juliana$$b50
000275763 7001_ $$aArock, Michel$$b51
000275763 7001_ $$00000-0003-0456-5095$$aValent, Peter$$b52
000275763 7001_ $$aSperr, Wolfgang R$$b53
000275763 7001_ $$aGotlib, Jason$$b54
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