U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Mast cell leukemia

MedGen UID:
44123
Concept ID:
C0023461
Neoplastic Process
Synonyms: Leukemia, Mast Cell; Leukemia, Mast-Cell; Leukemias, Mast-Cell; Mast Cell Leukemia; Mast-Cell Leukemia; Mast-Cell Leukemias
SNOMED CT: Mast cell leukemia (clinical) (110002002); Mast cell leukemia (128924002); Mast cell leukemia (110002002)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0020334
OMIM®: 154800
Orphanet: ORPHA98851

Definition

A very rare malignant systemic mastocytosis (SM) characterized by a huge infiltration of bone marrow, and often of blood, by abnormal mast cells (MC) which frequently manifests with organ dysfunction (liver, spleen, peritoneum, bones, and marrow). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMast cell leukemia
Follow this link to review classifications for Mast cell leukemia in Orphanet.

Professional guidelines

PubMed

Pardanani A
Am J Hematol 2021 Apr 1;96(4):508-525. Epub 2021 Feb 21 doi: 10.1002/ajh.26118. PMID: 33524167
Pardanani A
Am J Hematol 2019 Mar;94(3):363-377. Epub 2019 Jan 2 doi: 10.1002/ajh.25371. PMID: 30536695
Stankov K, Popovic S, Mikov M
Curr Pharm Des 2014;20(17):2849-80. doi: 10.2174/13816128113199990593. PMID: 23944364

Recent clinical studies

Etiology

Kennedy VE, Perkins C, Reiter A, Jawhar M, Lübke J, Kluin-Nelemans HC, Shomali W, Langford C, Abuel J, Hermine O, Niedoszytko M, Gorska A, Mital A, Bonadonna P, Zanotti R, Tanasi I, Mattsson M, Hagglund H, Triggiani M, Yavuz AS, Panse J, Christen D, Heizmann M, Shoumariyeh K, Müller S, Elena C, Malcovati L, Fiorelli N, Wortmann F, Vucinic V, Brockow K, Fokoloros C, Papageorgiou SG, Breynaert C, Bullens D, Doubek M, Ilerhaus A, Angelova-Fischer I, Solomianyi O, Várkonyi J, Sabato V, Rüfer A, Schug TD, Hermans MAW, Fortina AB, Caroppo F, Bumbea H, Gulen T, Hartmann K, Elberink HO, Schwaab J, Arock M, Valent P, Sperr WR, Gotlib J
Blood Adv 2023 May 9;7(9):1713-1724. doi: 10.1182/bloodadvances.2022008292. PMID: 36094848Free PMC Article
Pardanani A
Am J Hematol 2021 Apr 1;96(4):508-525. Epub 2021 Feb 21 doi: 10.1002/ajh.26118. PMID: 33524167
Laforgia M, Calabrò C, Scattone A, Laface C, Porcelli M, Gadaleta CD, Nardulli P, Ranieri G
Expert Opin Pharmacother 2020 Jun;21(9):1059-1069. Epub 2020 Mar 25 doi: 10.1080/14656566.2020.1744566. PMID: 32208985
Pardanani A
Am J Hematol 2019 Mar;94(3):363-377. Epub 2019 Jan 2 doi: 10.1002/ajh.25371. PMID: 30536695
Chiu A, Orazi A
Semin Diagn Pathol 2012 Feb;29(1):19-30. doi: 10.1053/j.semdp.2011.06.002. PMID: 22372203

Diagnosis

Beyens M, Elst J, van der Poorten ML, Van Gasse A, Toscano A, Verlinden A, Vermeulen K, Maes MB, Oude Elberink JNGH, Ebo D, Sabato V
Acta Clin Belg 2023 Aug;78(4):325-335. Epub 2022 Oct 19 doi: 10.1080/17843286.2022.2137631. PMID: 36259506
Leguit RJ, Wang SA, George TI, Tzankov A, Orazi A
Virchows Arch 2023 Jan;482(1):99-112. Epub 2022 Oct 10 doi: 10.1007/s00428-022-03423-3. PMID: 36214901
Kennedy VE, Perkins C, Reiter A, Jawhar M, Lübke J, Kluin-Nelemans HC, Shomali W, Langford C, Abuel J, Hermine O, Niedoszytko M, Gorska A, Mital A, Bonadonna P, Zanotti R, Tanasi I, Mattsson M, Hagglund H, Triggiani M, Yavuz AS, Panse J, Christen D, Heizmann M, Shoumariyeh K, Müller S, Elena C, Malcovati L, Fiorelli N, Wortmann F, Vucinic V, Brockow K, Fokoloros C, Papageorgiou SG, Breynaert C, Bullens D, Doubek M, Ilerhaus A, Angelova-Fischer I, Solomianyi O, Várkonyi J, Sabato V, Rüfer A, Schug TD, Hermans MAW, Fortina AB, Caroppo F, Bumbea H, Gulen T, Hartmann K, Elberink HO, Schwaab J, Arock M, Valent P, Sperr WR, Gotlib J
Blood Adv 2023 May 9;7(9):1713-1724. doi: 10.1182/bloodadvances.2022008292. PMID: 36094848Free PMC Article
Pardanani A
Am J Hematol 2021 Apr 1;96(4):508-525. Epub 2021 Feb 21 doi: 10.1002/ajh.26118. PMID: 33524167
Georgin-Lavialle S, Lhermitte L, Dubreuil P, Chandesris MO, Hermine O, Damaj G
Blood 2013 Feb 21;121(8):1285-95. Epub 2012 Dec 14 doi: 10.1182/blood-2012-07-442400. PMID: 23243287

Therapy

Bose P, Verstovsek S
Expert Rev Hematol 2021 Aug;14(8):687-696. Epub 2021 Aug 6 doi: 10.1080/17474086.2021.1959315. PMID: 34289787
Pardanani A
Am J Hematol 2021 Apr 1;96(4):508-525. Epub 2021 Feb 21 doi: 10.1002/ajh.26118. PMID: 33524167
Pardanani A
Am J Hematol 2019 Mar;94(3):363-377. Epub 2019 Jan 2 doi: 10.1002/ajh.25371. PMID: 30536695
Valent P, Akin C, Hartmann K, George TI, Sotlar K, Peter B, Gleixner KV, Blatt K, Sperr WR, Manley PW, Hermine O, Kluin-Nelemans HC, Arock M, Horny HP, Reiter A, Gotlib J
Ann Oncol 2017 Oct 1;28(10):2367-2376. doi: 10.1093/annonc/mdx290. PMID: 28945834Free PMC Article
Stankov K, Popovic S, Mikov M
Curr Pharm Des 2014;20(17):2849-80. doi: 10.2174/13816128113199990593. PMID: 23944364

Prognosis

Kennedy VE, Perkins C, Reiter A, Jawhar M, Lübke J, Kluin-Nelemans HC, Shomali W, Langford C, Abuel J, Hermine O, Niedoszytko M, Gorska A, Mital A, Bonadonna P, Zanotti R, Tanasi I, Mattsson M, Hagglund H, Triggiani M, Yavuz AS, Panse J, Christen D, Heizmann M, Shoumariyeh K, Müller S, Elena C, Malcovati L, Fiorelli N, Wortmann F, Vucinic V, Brockow K, Fokoloros C, Papageorgiou SG, Breynaert C, Bullens D, Doubek M, Ilerhaus A, Angelova-Fischer I, Solomianyi O, Várkonyi J, Sabato V, Rüfer A, Schug TD, Hermans MAW, Fortina AB, Caroppo F, Bumbea H, Gulen T, Hartmann K, Elberink HO, Schwaab J, Arock M, Valent P, Sperr WR, Gotlib J
Blood Adv 2023 May 9;7(9):1713-1724. doi: 10.1182/bloodadvances.2022008292. PMID: 36094848Free PMC Article
Pardanani A
Am J Hematol 2021 Apr 1;96(4):508-525. Epub 2021 Feb 21 doi: 10.1002/ajh.26118. PMID: 33524167
Pardanani A
Am J Hematol 2019 Mar;94(3):363-377. Epub 2019 Jan 2 doi: 10.1002/ajh.25371. PMID: 30536695
Stankov K, Popovic S, Mikov M
Curr Pharm Des 2014;20(17):2849-80. doi: 10.2174/13816128113199990593. PMID: 23944364
Ozdemir D, Dagdelen S, Erbas T
Am J Med Sci 2011 Nov;342(5):409-15. doi: 10.1097/MAJ.0b013e3182121131. PMID: 21629042

Clinical prediction guides

Proaño-Pérez E, Ollé L, Guo Y, Aparicio C, Guerrero M, Muñoz-Cano R, Martin M
Int J Mol Sci 2023 Feb 9;24(4) doi: 10.3390/ijms24043515. PMID: 36834926Free PMC Article
Bose P, Verstovsek S
Expert Rev Hematol 2021 Aug;14(8):687-696. Epub 2021 Aug 6 doi: 10.1080/17474086.2021.1959315. PMID: 34289787
Jawhar M, Schwaab J, Meggendorfer M, Naumann N, Horny HP, Sotlar K, Haferlach T, Schmitt K, Fabarius A, Valent P, Hofmann WK, Cross NCP, Metzgeroth G, Reiter A
Haematologica 2017 Jun;102(6):1035-1043. Epub 2017 Mar 2 doi: 10.3324/haematol.2017.163964. PMID: 28255023Free PMC Article
Stankov K, Popovic S, Mikov M
Curr Pharm Des 2014;20(17):2849-80. doi: 10.2174/13816128113199990593. PMID: 23944364
Valent P, Sotlar K, Horny HP
Leuk Lymphoma 2011 May;52(5):740-4. Epub 2011 Jan 24 doi: 10.3109/10428194.2010.550072. PMID: 21261503

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...