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Hematologic neoplasm

MedGen UID:
138213
•Concept ID:
C0376545
•
Neoplastic Process
Synonyms: Hematologic cancer; Hematological malignancies; Hematological neoplasm
SNOMED CT: Hematologic neoplasm (129154003); Hematologic malignancy (269475001)
 
Gene (location): H3C2 (6p22.2)
 
HPO: HP:0004377

Definition

Neoplasms located in the blood and blood-forming tissue (the bone marrow and lymphatic tissue). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHematologic neoplasm

Conditions with this feature

Platelet disorder, undefined
MedGen UID:
401405
•Concept ID:
C1868258
•
Disease or Syndrome
See: Condition Record
Platelet disorder, undefined

Professional guidelines

PubMed

Personalized Management Strategies in Mast Cell Disorders: ECNM-AIM User's Guide for Daily Clinical Practice.
Valent P, Hartmann K, Schwaab J, Alvarez-Twose I, Brockow K, Bonadonna P, Hermine O, Niedoszytko M, Carter MC, Hoermann G, Sperr WR, Butterfield JH, Ustun C, Zanotti R, Radia DH, Castells M, Triggiani M, Schwartz LB, Orfao A, George TI, Sotlar K, Gotlib J, Reiter A, Horny HP, Arock M, Akin C, Metcalfe DD
J Allergy Clin Immunol Pract 2022 Aug;10(8):1999-2012.e6. Epub 2022 Mar 25 doi: 10.1016/j.jaip.2022.03.007. PMID: 35342031
Systemic Mastocytosis: Advances in Diagnosis and Current Management.
Pullarkat ST, Wu W, Pullarkat V
Cancer Treat Res 2021;181:167-178. doi: 10.1007/978-3-030-78311-2_10. PMID: 34626361
New developments in diagnosis, prognostication, and treatment of advanced systemic mastocytosis.
Reiter A, George TI, Gotlib J
Blood 2020 Apr 16;135(16):1365-1376. doi: 10.1182/blood.2019000932. PMID: 32106312

Curated

Thiopurine methyltransferase (TPMT) genotyping to predict myelosuppression risk

Recent clinical studies

Etiology

New Insights into the Pathogenesis of Mastocytosis: Emerging Concepts in Diagnosis and Therapy.
Valent P, Akin C, Sperr WR, Horny HP, Arock M, Metcalfe DD, Galli SJ
Annu Rev Pathol 2023 Jan 24;18:361-386. Epub 2022 Oct 21 doi: 10.1146/annurev-pathmechdis-031521-042618. PMID: 36270293
Update on diagnostic approaches and therapeutic strategies in systemic mastocytosis.
Radia DH, Moonim MT
Best Pract Res Clin Haematol 2022 Jun;35(2):101380. Epub 2022 Sep 14 doi: 10.1016/j.beha.2022.101380. PMID: 36333068
ANKRD26-Related Thrombocytopenia and Predisposition to Myeloid Neoplasms.
Sullivan MJ, Palmer EL, Botero JP
Curr Hematol Malig Rep 2022 Oct;17(5):105-112. Epub 2022 Jun 25 doi: 10.1007/s11899-022-00666-4. PMID: 35751752
Clonal hematopoiesis in hematopoietic stem cell transplantation.
Wilk CM, Manz MG, Boettcher S
Curr Opin Hematol 2021 Mar 1;28(2):94-100. doi: 10.1097/MOH.0000000000000631. PMID: 33394723
Clinical Implications of Clonal Hematopoiesis.
Steensma DP
Mayo Clin Proc 2018 Aug;93(8):1122-1130. Epub 2018 Jul 4 doi: 10.1016/j.mayocp.2018.04.002. PMID: 30078412

Diagnosis

New Insights into the Pathogenesis of Mastocytosis: Emerging Concepts in Diagnosis and Therapy.
Valent P, Akin C, Sperr WR, Horny HP, Arock M, Metcalfe DD, Galli SJ
Annu Rev Pathol 2023 Jan 24;18:361-386. Epub 2022 Oct 21 doi: 10.1146/annurev-pathmechdis-031521-042618. PMID: 36270293
The international consensus classification of mastocytosis and related entities.
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
Personalized Management Strategies in Mast Cell Disorders: ECNM-AIM User's Guide for Daily Clinical Practice.
Valent P, Hartmann K, Schwaab J, Alvarez-Twose I, Brockow K, Bonadonna P, Hermine O, Niedoszytko M, Carter MC, Hoermann G, Sperr WR, Butterfield JH, Ustun C, Zanotti R, Radia DH, Castells M, Triggiani M, Schwartz LB, Orfao A, George TI, Sotlar K, Gotlib J, Reiter A, Horny HP, Arock M, Akin C, Metcalfe DD
J Allergy Clin Immunol Pract 2022 Aug;10(8):1999-2012.e6. Epub 2022 Mar 25 doi: 10.1016/j.jaip.2022.03.007. PMID: 35342031
Avapritinib for Systemic Mastocytosis.
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
New developments in diagnosis, prognostication, and treatment of advanced systemic mastocytosis.
Reiter A, George TI, Gotlib J
Blood 2020 Apr 16;135(16):1365-1376. doi: 10.1182/blood.2019000932. PMID: 32106312

Therapy

Blinatumomab in Practice.
Lantz J, Pham N, Jones C, Reed D, El Chaer F, Keng M
Curr Hematol Malig Rep 2024 Feb;19(1):1-8. Epub 2023 Dec 7 doi: 10.1007/s11899-023-00714-7. PMID: 38060085
Avapritinib for advanced systemic mastocytosis.
Gotlib J, Reiter A, DeAngelo DJ
Blood 2022 Oct 13;140(15):1667-1673. doi: 10.1182/blood.2021014612. PMID: 35877999
Safety and efficacy of avapritinib in advanced systemic mastocytosis: the phase 1 EXPLORER trial.
DeAngelo DJ, Radia DH, George TI, Robinson WA, Quiery AT, Drummond MW, Bose P, Hexner EO, Winton EF, Horny HP, Tugnait M, Schmidt-Kittler O, Evans EK, Lin HM, Mar BG, Verstovsek S, Deininger MW, Gotlib J
Nat Med 2021 Dec;27(12):2183-2191. Epub 2021 Dec 6 doi: 10.1038/s41591-021-01538-9. PMID: 34873347Free PMC Article
Avapritinib for Systemic Mastocytosis.
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
New developments in diagnosis, prognostication, and treatment of advanced systemic mastocytosis.
Reiter A, George TI, Gotlib J
Blood 2020 Apr 16;135(16):1365-1376. doi: 10.1182/blood.2019000932. PMID: 32106312

Prognosis

Blinatumomab in Practice.
Lantz J, Pham N, Jones C, Reed D, El Chaer F, Keng M
Curr Hematol Malig Rep 2024 Feb;19(1):1-8. Epub 2023 Dec 7 doi: 10.1007/s11899-023-00714-7. PMID: 38060085
New Insights into the Pathogenesis of Mastocytosis: Emerging Concepts in Diagnosis and Therapy.
Valent P, Akin C, Sperr WR, Horny HP, Arock M, Metcalfe DD, Galli SJ
Annu Rev Pathol 2023 Jan 24;18:361-386. Epub 2022 Oct 21 doi: 10.1146/annurev-pathmechdis-031521-042618. PMID: 36270293
Avapritinib for Systemic Mastocytosis.
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
New developments in diagnosis, prognostication, and treatment of advanced systemic mastocytosis.
Reiter A, George TI, Gotlib J
Blood 2020 Apr 16;135(16):1365-1376. doi: 10.1182/blood.2019000932. PMID: 32106312
Relevant updates in systemic mastocytosis.
Coltoff A, Mascarenhas J
Leuk Res 2019 Jun;81:10-18. Epub 2019 Apr 4 doi: 10.1016/j.leukres.2019.04.001. PMID: 30978435

Clinical prediction guides

Update on diagnostic approaches and therapeutic strategies in systemic mastocytosis.
Radia DH, Moonim MT
Best Pract Res Clin Haematol 2022 Jun;35(2):101380. Epub 2022 Sep 14 doi: 10.1016/j.beha.2022.101380. PMID: 36333068
Personalized Management Strategies in Mast Cell Disorders: ECNM-AIM User's Guide for Daily Clinical Practice.
Valent P, Hartmann K, Schwaab J, Alvarez-Twose I, Brockow K, Bonadonna P, Hermine O, Niedoszytko M, Carter MC, Hoermann G, Sperr WR, Butterfield JH, Ustun C, Zanotti R, Radia DH, Castells M, Triggiani M, Schwartz LB, Orfao A, George TI, Sotlar K, Gotlib J, Reiter A, Horny HP, Arock M, Akin C, Metcalfe DD
J Allergy Clin Immunol Pract 2022 Aug;10(8):1999-2012.e6. Epub 2022 Mar 25 doi: 10.1016/j.jaip.2022.03.007. PMID: 35342031
Safety and efficacy of avapritinib in advanced systemic mastocytosis: the phase 1 EXPLORER trial.
DeAngelo DJ, Radia DH, George TI, Robinson WA, Quiery AT, Drummond MW, Bose P, Hexner EO, Winton EF, Horny HP, Tugnait M, Schmidt-Kittler O, Evans EK, Lin HM, Mar BG, Verstovsek S, Deininger MW, Gotlib J
Nat Med 2021 Dec;27(12):2183-2191. Epub 2021 Dec 6 doi: 10.1038/s41591-021-01538-9. PMID: 34873347Free PMC Article
Avapritinib for Systemic Mastocytosis.
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
New developments in diagnosis, prognostication, and treatment of advanced systemic mastocytosis.
Reiter A, George TI, Gotlib J
Blood 2020 Apr 16;135(16):1365-1376. doi: 10.1182/blood.2019000932. PMID: 32106312

Recent systematic reviews

Extracorporeal membrane oxygenation in patients with hematologic malignancies: a systematic review and meta-analysis.
Sim JJL, Mitra S, Ling RR, Tan CS, Fan BE, MacLaren G, Ramanathan K
Ann Hematol 2022 Jul;101(7):1395-1406. Epub 2022 May 27 doi: 10.1007/s00277-022-04855-2. PMID: 35622097
Prognostic and therapeutic value of somatic mutations in diffuse large B-cell lymphoma: A systematic review.
Lopez-Santillan M, Lopez-Lopez E, Alvarez-Gonzalez P, Martinez G, Arzuaga-Mendez J, Ruiz-Diaz I, Guerra-Merino I, Gutierrez-Camino A, Martin-Guerrero I
Crit Rev Oncol Hematol 2021 Sep;165:103430. Epub 2021 Jul 30 doi: 10.1016/j.critrevonc.2021.103430. PMID: 34339834
Laboratory biomarkers for venous thromboembolism risk in patients with hematologic malignancies: A review.
Samuelson Bannow BT, Konkle BA
Thromb Res 2018 Mar;163:138-145. Epub 2018 Jan 31 doi: 10.1016/j.thromres.2018.01.037. PMID: 29407626Free PMC Article
Anterior ischemic optic neuropathy and hematologic malignancy: a systematic review of case reports and case series.
Sousa DC, Rodrigues FB, Duarte G, Campos F, Pinto F, Vaz-Carneiro A
Can J Ophthalmol 2016 Dec;51(6):459-466. Epub 2016 Sep 14 doi: 10.1016/j.jcjo.2016.05.018. PMID: 27938958

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.

    Curated

    • Nguyen et al., 2011
      Thiopurine methyltransferase (TPMT) genotyping to predict myelosuppression risk

    Consumer resources

    Reviews

    Related information

    • ClinVar
      Related medical variations
    • Gene
      Related information in NCBI Gene
    • GTR
      Related information in GTR
    • GTR(Clinical)
      Clinical tests in GTR
    • MeSH
      Related Medical Subject Headings
    • OMIM(Genes)
      OMIM records containing genes associated with phenotypes registered in MedGen
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

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