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Systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disease

MedGen UID:
226985
Concept ID:
C1301365
Neoplastic Process
Synonyms: SM-AHN; SM-AHNMD; SMAHN; Systemic mastocytosis with AHNMD; systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disease; systemic mastocytosis with an associated haematological neoplasm; systemic mastocytosis with an associated haematological neoplasm (SM-AHN); Systemic Mastocytosis with an Associated Hematological Neoplasm; systemic mastocytosis with an associated hematological neoplasm; Systemic Mastocytosis with an Associated Hematological Neoplasm (SM-AHN); systemic mastocytosis with an associated hematological neoplasm (SM-AHN); systemic mastocytosis with associated clonal haematological non-mast cell lineage disease; systemic mastocytosis with associated clonal haematological non-mast-cell lineage disease; Systemic mastocytosis with associated clonal hematological non-mast cell lineage disease; systemic mastocytosis with associated clonal hematological non-mast cell lineage disease; Systemic Mastocytosis with Associated Clonal Hematological non-Mast-Cell Lineage Disease; systemic mastocytosis with associated clonal hematological non-mast-cell lineage disease; Systemic mastocytosis with associated clonal, hematologic non-mast-cell lineage disease; systemic mastocytosis with associated clonal, hematologic non-mast-cell lineage disease; systemic mastocytosis with associated clonal, hematologic non-mast-cell lineage disease (morphologic abnormality); systemic mastocytosis with associated hematologic neoplasm; Systemic mastocytosis with associated hematologic neoplasm; Systemic mastocytosis with associated hematological clonal non-mast cell disorder
SNOMED CT: Systemic mastocytosis with associated clonal hematological non-mast cell lineage disease (397015000); Systemic mastocytosis with associated clonal, hematologic non-mast-cell lineage disease (397357000); Systemic mastocytosis with AHNMD (397357000); Systemic mastocytosis with associated hematological clonal non-mast cell disorder (397357000)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0020332
Orphanet: ORPHA98849

Definition

An advanced form of systemic mastocytosis (SM) characterized by the abnormal accumulation of neoplastic mast cells (MCs) in one or more extracutaneous organs, mainly the bone marrow, associated with another hematologic neoplasm of non MC nature. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSystemic mastocytosis with an associated clonal hematologic non-mast cell lineage disease
Follow this link to review classifications for Systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disease in Orphanet.

Professional guidelines

PubMed

Azaña JM, Torrelo A, Matito A
Actas Dermosifiliogr 2016 Jan-Feb;107(1):15-22. Epub 2015 Oct 30 doi: 10.1016/j.ad.2015.09.009. PMID: 26525106
Verstovsek S
Eur J Haematol 2013 Feb;90(2):89-98. doi: 10.1111/ejh.12043. PMID: 23181448Free PMC Article
Valent P, Horny HP, Escribano L, Longley BJ, Li CY, Schwartz LB, Marone G, Nuñez R, Akin C, Sotlar K, Sperr WR, Wolff K, Brunning RD, Parwaresch RM, Austen KF, Lennert K, Metcalfe DD, Vardiman JW, Bennett JM
Leuk Res 2001 Jul;25(7):603-25. doi: 10.1016/s0145-2126(01)00038-8. PMID: 11377686

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Systemic Mastocytosis, 2024

Recent clinical studies

Etiology

Ustun C, Arock M, Kluin-Nelemans HC, Reiter A, Sperr WR, George T, Horny HP, Hartmann K, Sotlar K, Damaj G, Hermine O, Verstovsek S, Metcalfe DD, Gotlib J, Akin C, Valent P
Haematologica 2016 Oct;101(10):1133-1143. doi: 10.3324/haematol.2016.146563. PMID: 27694501
Azaña JM, Torrelo A, Matito A
Actas Dermosifiliogr 2016 Jan-Feb;107(1):15-22. Epub 2015 Oct 30 doi: 10.1016/j.ad.2015.09.009. PMID: 26525106
Cohen SS, Skovbo S, Vestergaard H, Kristensen T, Møller M, Bindslev-Jensen C, Fryzek JP, Broesby-Olsen S
Br J Haematol 2014 Aug;166(4):521-8. Epub 2014 Apr 25 doi: 10.1111/bjh.12916. PMID: 24761987
Pagano L, Valentini CG, Caira M, Rondoni M, Van Lint MT, Candoni A, Allione B, Cattaneo C, Marbello L, Caramatti C, Pogliani EM, Iannitto E, Giona F, Ferrara F, Invernizzi R, Fanci R, Lunghi M, Fianchi L, Sanpaolo G, Stefani PM, Pulsoni A, Martinelli G, Leone G, Musto P
Int J Hematol 2008 Dec;88(5):483-488. Epub 2008 Nov 26 doi: 10.1007/s12185-008-0166-4. PMID: 19034614
Simon J, Lortholary O, Caillat-Vigneron N, Raphaël M, Martin A, Brière J, Barète S, Hermine O, Casassus P; Group AFIRMM (Association française pour les initiatives de recherche sur le mastocyte et les mastocytoses)
Pathol Biol (Paris) 2004 Jun;52(5):294-9. doi: 10.1016/j.patbio.2004.04.012. PMID: 15217717

Diagnosis

Azaña JM, Torrelo A, Matito A
Actas Dermosifiliogr 2016 Jan-Feb;107(1):15-22. Epub 2015 Oct 30 doi: 10.1016/j.ad.2015.09.009. PMID: 26525106
Verstovsek S
Eur J Haematol 2013 Feb;90(2):89-98. doi: 10.1111/ejh.12043. PMID: 23181448Free PMC Article
Metcalfe DD
Novartis Found Symp 2005;271:232-42; discussion 242-9. PMID: 16605139
Pullarkat VA, Bueso-Ramos C, Lai R, Kroft S, Wilson CS, Pullarkat ST, Bu X, Thein M, Lee M, Brynes RK
Am J Hematol 2003 May;73(1):12-7. doi: 10.1002/ajh.10322. PMID: 12701114
Sperr WR, Horny HP, Valent P
Int Arch Allergy Immunol 2002 Feb;127(2):140-2. doi: 10.1159/000048186. PMID: 11919425

Therapy

Marton I, Krenács L, Bagdi E, Bakos A, Demeter J, Borbényi Z
Pathol Oncol Res 2016 Apr;22(2):293-9. Epub 2015 Nov 6 doi: 10.1007/s12253-015-9948-1. PMID: 26545382
Ustun C, DeRemer DL, Akin C
Leuk Res 2011 Sep;35(9):1143-52. Epub 2011 Jun 8 doi: 10.1016/j.leukres.2011.05.006. PMID: 21641642
Du S, Rashidi HH, Le DT, Kipps TJ, Broome HE, Wang HY
Int J Clin Exp Pathol 2010 Apr 23;3(4):448-57. PMID: 20490336Free PMC Article
Pagano L, Valentini CG, Caira M, Rondoni M, Van Lint MT, Candoni A, Allione B, Cattaneo C, Marbello L, Caramatti C, Pogliani EM, Iannitto E, Giona F, Ferrara F, Invernizzi R, Fanci R, Lunghi M, Fianchi L, Sanpaolo G, Stefani PM, Pulsoni A, Martinelli G, Leone G, Musto P
Int J Hematol 2008 Dec;88(5):483-488. Epub 2008 Nov 26 doi: 10.1007/s12185-008-0166-4. PMID: 19034614
Simon J, Lortholary O, Caillat-Vigneron N, Raphaël M, Martin A, Brière J, Barète S, Hermine O, Casassus P; Group AFIRMM (Association française pour les initiatives de recherche sur le mastocyte et les mastocytoses)
Pathol Biol (Paris) 2004 Jun;52(5):294-9. doi: 10.1016/j.patbio.2004.04.012. PMID: 15217717

Prognosis

Ustun C, Arock M, Kluin-Nelemans HC, Reiter A, Sperr WR, George T, Horny HP, Hartmann K, Sotlar K, Damaj G, Hermine O, Verstovsek S, Metcalfe DD, Gotlib J, Akin C, Valent P
Haematologica 2016 Oct;101(10):1133-1143. doi: 10.3324/haematol.2016.146563. PMID: 27694501
Azaña JM, Torrelo A, Matito A
Actas Dermosifiliogr 2016 Jan-Feb;107(1):15-22. Epub 2015 Oct 30 doi: 10.1016/j.ad.2015.09.009. PMID: 26525106
Wang SA, Hutchinson L, Tang G, Chen SS, Miron PM, Huh YO, Jones DM, Bueso-Ramos C, Verstovsek S, Medeiros LJ, Miranda RN
Am J Hematol 2013 Mar;88(3):219-24. doi: 10.1002/ajh.23380. PMID: 23440662Free PMC Article
Verstovsek S
Eur J Haematol 2013 Feb;90(2):89-98. doi: 10.1111/ejh.12043. PMID: 23181448Free PMC Article
Sperr WR, Horny HP, Valent P
Int Arch Allergy Immunol 2002 Feb;127(2):140-2. doi: 10.1159/000048186. PMID: 11919425

Clinical prediction guides

Djelbani-Ahmed S, Chandesris MO, Mekinian A, Canioni D, Brouzes C, Hanssens K, Pop G, Durieu I, Durupt S, Grosbois B, Besnard S, Tournilhac O, Beyne-Rauzy O, Agapé P, Delmer A, Ranta D, Jeandel PY, Georgin-Lavialle S, Frenzel L, Damaj G, Eder V, Lortholary O, Hermine O, Fain O, Soussan M
Eur J Nucl Med Mol Imaging 2015 Dec;42(13):2013-20. Epub 2015 Jul 5 doi: 10.1007/s00259-015-3117-3. PMID: 26140850
Gasljevic G, Grcar-Kuzmanov B, Grosel A, Sever M, Gazic B, Kloboves-Prevodnik V
Diagn Pathol 2015 Mar 14;10:5. doi: 10.1186/s13000-015-0235-y. PMID: 25881198Free PMC Article
Tefferi A, Levine RL, Lim KH, Abdel-Wahab O, Lasho TL, Patel J, Finke CM, Mullally A, Li CY, Pardanani A, Gilliland DG
Leukemia 2009 May;23(5):900-4. Epub 2009 Mar 5 doi: 10.1038/leu.2009.37. PMID: 19262599Free PMC Article
Simon J, Lortholary O, Caillat-Vigneron N, Raphaël M, Martin A, Brière J, Barète S, Hermine O, Casassus P; Group AFIRMM (Association française pour les initiatives de recherche sur le mastocyte et les mastocytoses)
Pathol Biol (Paris) 2004 Jun;52(5):294-9. doi: 10.1016/j.patbio.2004.04.012. PMID: 15217717
Pullarkat VA, Bueso-Ramos C, Lai R, Kroft S, Wilson CS, Pullarkat ST, Bu X, Thein M, Lee M, Brynes RK
Am J Hematol 2003 May;73(1):12-7. doi: 10.1002/ajh.10322. PMID: 12701114

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.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Systemic Mastocytosis, 2024

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