Mast cell leukemia: clinical and molecular features and survival outcomes of patients in the ECNM Registry

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Kennedy, Vanessa E.Univ Calif San Francisco, San Francisco, CA USA. (author)

Mast cell leukemia : clinical and molecular features and survival outcomes of patients in the ECNM Registry

Article/chapterEnglish2023

Publisher, publication year, extent ...

2022-09-12
American Society of Hematology,2023
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LIBRIS-ID:oai:DiVA.org:uu-508419
https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-508419URI
https://doi.org/10.1182/bloodadvances.2022008292DOI
http://kipublications.ki.se/Default.aspx?queryparsed=id:153083084URI

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Language:English
Summary in:English

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SwePubSwePub

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Subject category:ref swepub-contenttype
Subject category:art swepub-publicationtype

Notes

Mast 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.

Subject headings and genre

MEDICIN OCH HÄLSOVETENSKAP Klinisk medicin Hematologi hsv//swe
MEDICAL AND HEALTH SCIENCES Clinical Medicine Hematology hsv//eng
MEDICIN OCH HÄLSOVETENSKAP Klinisk medicin Cancer och onkologi hsv//swe
MEDICAL AND HEALTH SCIENCES Clinical Medicine Cancer and Oncology hsv//eng

Added entries (persons, corporate bodies, meetings, titles ...)

Perkins, CeceliaStanford Univ, Sch Med, Stanford Canc Inst, Stanford, CA 94305 USA. (author)
Reiter, AndreasHeidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany. (author)
Jawhar, MohamadHeidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany. (author)
Luebke, JohannesHeidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany. (author)
Kluin-Nelemans, Hanneke C.Univ Groningen, Univ Med Ctr Groningen, Groningen, Netherlands. (author)
Shomali, WilliamStanford Univ, Sch Med, Stanford Canc Inst, Stanford, CA 94305 USA. (author)
Langford, CherylStanford Univ, Sch Med, Stanford Canc Inst, Stanford, CA 94305 USA. (author)
Abuel, JustinStanford Univ, Sch Med, Stanford Canc Inst, Stanford, CA 94305 USA. (author)
Hermine, OlivierUniv Paris Sorbonne, Hop Necker, AP HP, Imagine Inst,INSERM,U1163,Ctr Natl Reference Mast, Paris, France. (author)
Niedoszytko, MarekMed Univ Gdansk, Dept Allergol, Gdansk, Poland. (author)
Gorska, AleksandraMed Univ Gdansk, Dept Allergol, Gdansk, Poland. (author)
Mital, AndrzejMed Univ Gdansk, Dept Hematol, Gdansk, Poland. (author)
Bonadonna, PatriziaVerona Univ Hosp, Sect Hematol, Dept Med, Verona, Italy. (author)
Zanotti, RobertaVerona Univ Hosp, Sect Hematol, Dept Med, Verona, Italy. (author)
Tanasi, IlariaVerona Univ Hosp, Sect Hematol, Dept Med, Verona, Italy. (author)
Mattsson, MattiasUppsala universitet,Hematologi,Institutionen för immunologi, genetik och patologi,Uppsala Univ, Univ Uppsala Hosp, Dept Hematol, Uppsala, Sweden.(Swepub:uu)matma431 (author)
Hägglund, HansUppsala universitet,Hematologi,Institutionen för immunologi, genetik och patologi,Uppsala Univ, Univ Uppsala Hosp, Dept Hematol, Uppsala, Sweden.(Swepub:uu)hanha689 (author)
Triggiani, MassimoUniv Salerno, Div Allergy & Clin Immunol, Salerno, Italy. (author)
Yavuz, Akif SelimIstanbul Univ, Istanbul Med Sch, Div Hematol, Istanbul, Turkiye. (author)
Panse, JensUniv Hosp RWTH Aachen, Dept Oncol Haematol Haemostaseol & Stem Cell Tran, Aachen, Germany.;Ctr Integrated Oncol, Aachen, Germany. (author)
Christen, DeborahUniv Hosp RWTH Aachen, Dept Oncol Haematol Haemostaseol & Stem Cell Tran, Aachen, Germany.;Ctr Integrated Oncol, Aachen, Germany. (author)
Heizmann, MarcKantonsspital Aarau AG, Univ Clin Med, Div Oncol Haematol & Transfus Med, Aarau, Switzerland. (author)
Shoumariyeh, KhalidUniv Freiburg, Fac Med, Med Ctr, Dept Med 1, Freiburg, Germany.;German Canc Consortium DKTK, Partner Site Freiburg, Freiburg, Germany. (author)
Mueller, SabineUniv Frieburg, Dept Dermatol, Med Ctr, Fac Med, Freiburg, Germany. (author)
Elena, ChiaraFdn IRCCS Policlin San Matteo, Hematol Unit, Pavia, Italy. (author)
Malcovati, LucaFdn IRCCS Policlin San Matteo, Hematol Unit, Pavia, Italy. (author)
Fiorelli, NicolasFdn IRCCS Policlin San Matteo, Hematol Unit, Pavia, Italy. (author)
Wortmann, FriederikeUniv Klinikum Schleswig Holstein, Klin Hamatol & Onkol, Lubeck, Germany. (author)
Vucinic, VladanUniv Klinikum Leipzig AoR, Leipzig, Germany. (author)
Brockow, KnutTech Univ Munich, Sch Med, Dept Dermatol & Allergy Biederstein, Munich, Germany. (author)
Fokoloros, ChristosUniv Athens, Attikon Univ Hosp, Dept Dermatol & Venereol 2, Mastocytosis Clin,Allergy Unit, Athens, Greece.;Univ Athens, Attikon Univ Hosp, Propaedeut Dept Internal Med 2, Athens, Greece.;Univ Athens, Attikon Univ Hosp, Res Inst, Hematol Unit, Athens, Greece. (author)
Papageorgiou, Sotirios G.Univ Athens, Attikon Univ Hosp, Dept Dermatol & Venereol 2, Mastocytosis Clin,Allergy Unit, Athens, Greece.;Univ Athens, Attikon Univ Hosp, Propaedeut Dept Internal Med 2, Athens, Greece.;Univ Athens, Attikon Univ Hosp, Res Inst, Hematol Unit, Athens, Greece. (author)
Breynaert, ChristineKatholieke Univ Leuven, Dept Microbiol Immunol & Transplantat, Allergy & Clin Immunol Res Grp, Leuven, Belgium.;Univ Hosp Leuven, MASTeL, Leuven, Belgium. (author)
Bullens, DominiqueKatholieke Univ Leuven, Dept Microbiol Immunol & Transplantat, Allergy & Clin Immunol Res Grp, Leuven, Belgium.;Univ Hosp Leuven, MASTeL, Leuven, Belgium. (author)
Doubek, MichaelBrno Univ Hosp, Brno, Czech Republic.;Fac Med, Brno, Czech Republic. (author)
Ilerhaus, AnjaUniklin Koln, Klin Dermatol & Venerol, Cologne, Germany. (author)
Angelova-Fischer, IrenaKepler Univ Hosp, Linz, Austria. (author)
Solomianyi, OleksiiKepler Univ Hosp, Linz, Austria. (author)
Varkonyi, JuditSemmelweis Univ, Dept Hematol, Budapest, Hungary. (author)
Sabato, VitoUniv Antwerp, Dept Immunol Allergy & Rheumatol, Campus Drie Eiken, Antwerp, Belgium. (author)
Ruefer, AxelLuzerner Kantonsspital, Luzern, Switzerland. (author)
Schug, Tanja DanielaMed Univ Graz, Univ Klin Dermatol, Graz, Austria. (author)
Hermans, Maud A. W.Erasmus MC, Sect Allergy & Clin Immunol, Dept Internal Med, Rotterdam, Netherlands. (author)
Fortina, Anna BelloniUniv Padov, Azienda Osped, Pediat Dermatol, Internal Med, Padua, Italy. (author)
Caroppo, FrancescaUniv Padov, Azienda Osped, Pediat Dermatol, Internal Med, Padua, Italy. (author)
Bumbea, HoriaCarol Davila Univ Med, Emergency Univ Hosp, Dept Hematol, Bucharest, Romania. (author)
Gulen, TheoKarolinska Univ Hosp Huddinge, Dept Resp Med & Allergy, Stockholm, Sweden.;Karolinska Inst, Dept Med Solna, Stockholm, Sweden. (author)
Hartmann, KarinUniv Hosp Basel, Dept Dermatol, Div Allergy, Basel, Switzerland.;Univ Hosp Basel, Dept Biomed, Div Allergy, Basel, Switzerland.;Univ Basel, Basel, Switzerland. (author)
Elberink, Hanneke OudeUniv Groningen, Univ Med Ctr Groningen, Groningen, Netherlands. (author)
Schwaab, JulianaHeidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany. (author)
Arock, MichelHop La Pitie Salpetriere, Lab Hematol, Paris, France. (author)
Valent, PeterMed Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria. (author)
Sperr, Wolfgang R.Med Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria. (author)
Gotlib, JasonStanford Univ, Sch Med, Stanford Canc Inst, Stanford, CA 94305 USA. (author)
Univ Calif San Francisco, San Francisco, CA USA.Stanford Univ, Sch Med, Stanford Canc Inst, Stanford, CA 94305 USA. (creator_code:org_t)

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In:Blood Advances: American Society of Hematology7:9, s. 1713-17242473-95292473-9537

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By the author/editor: Kennedy, Vanessa ...; Perkins, Cecelia; Reiter, Andreas; Jawhar, Mohamad; Luebke, Johannes; Kluin-Nelemans, ...; show more...; Shomali, William; Langford, Cheryl; Abuel, Justin; Hermine, Olivier; Niedoszytko, Mar ...; Gorska, Aleksand ...; Mital, Andrzej; Bonadonna, Patri ...; Zanotti, Roberta; Tanasi, Ilaria; Mattsson, Mattia ...; Hägglund, Hans; Triggiani, Massi ...; Yavuz, Akif Seli ...; Panse, Jens; Christen, Debora ...; Heizmann, Marc; Shoumariyeh, Kha ...; Mueller, Sabine; Elena, Chiara; Malcovati, Luca; Fiorelli, Nicola ...; Wortmann, Friede ...; Vucinic, Vladan; Brockow, Knut; Fokoloros, Chris ...; Papageorgiou, So ...; Breynaert, Chris ...; Bullens, Dominiq ...; Doubek, Michael; Ilerhaus, Anja; Angelova-Fischer ...; Solomianyi, Olek ...; Varkonyi, Judit; Sabato, Vito; Ruefer, Axel; Schug, Tanja Dan ...; Hermans, Maud A. ...; Fortina, Anna Be ...; Caroppo, Frances ...; Bumbea, Horia; Gulen, Theo; Hartmann, Karin; Elberink, Hannek ...; Schwaab, Juliana; Arock, Michel; Valent, Peter; Sperr, Wolfgang ...; Gotlib, Jason; show less...

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