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Chronic Eosinophilic Leukemia with FIP1L1-PDGFRA

MedGen UID:
927323
•Concept ID:
C4289791
•
Neoplastic Process
Synonym: Chronic Eosinophilic Leukemia with FIP1L1::PDGFRA

Definition

Chronic eosinophilic leukemia characterized by the rearrangement of the PDGFRA gene, most often resulting in the formation of FIP1L1-PDGFRA fusion transcripts. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChronic Eosinophilic Leukemia with FIP1L1-PDGFRA

Professional guidelines

PubMed

World Health Organization-defined eosinophilic disorders: 2019 update on diagnosis, risk stratification, and management.
Shomali W, Gotlib J
Am J Hematol 2019 Oct;94(10):1149-1167. doi: 10.1002/ajh.25617. PMID: 31423623
Management of Hypereosinophilic Syndromes.
Roufosse F
Immunol Allergy Clin North Am 2015 Aug;35(3):561-75. doi: 10.1016/j.iac.2015.05.006. PMID: 26209900
FIP1L1-PDGFR alpha, a therapeutic target for the treatment of chronic eosinophilic leukemia.
Cools J
Verh K Acad Geneeskd Belg 2005;67(3):169-76. PMID: 16089297

Recent clinical studies

Etiology

World Health Organization-defined eosinophilic disorders: 2019 update on diagnosis, risk stratification, and management.
Shomali W, Gotlib J
Am J Hematol 2019 Oct;94(10):1149-1167. doi: 10.1002/ajh.25617. PMID: 31423623
Hypereosinophilic Syndrome.
Curtis C, Ogbogu P
Clin Rev Allergy Immunol 2016 Apr;50(2):240-51. doi: 10.1007/s12016-015-8506-7. PMID: 26475367
Pathogenesis, classification, and therapy of eosinophilia and eosinophil disorders.
Valent P
Blood Rev 2009 Jul;23(4):157-65. Epub 2009 Feb 25 doi: 10.1016/j.blre.2009.01.001. PMID: 19246139
KIT D816V-associated systemic mastocytosis with eosinophilia and FIP1L1/PDGFRA-associated chronic eosinophilic leukemia are distinct entities.
Maric I, Robyn J, Metcalfe DD, Fay MP, Carter M, Wilson T, Fu W, Stoddard J, Scott L, Hartsell M, Kirshenbaum A, Akin C, Nutman TB, Noel P, Klion AD
J Allergy Clin Immunol 2007 Sep;120(3):680-7. Epub 2007 Jul 12 doi: 10.1016/j.jaci.2007.05.024. PMID: 17628645
FIP1L1-PDGFRA positive chronic eosinophilic leukemia in Tunisian patients.
Menif S, Omri H, Hafsia R, Ben Romdhane N, Turki S, Meddeb B, Dellagi K
Pathol Biol (Paris) 2007 Jun;55(5):242-5. Epub 2006 Nov 29 doi: 10.1016/j.patbio.2006.10.002. PMID: 17137731

Diagnosis

World Health Organization-defined eosinophilic disorders: 2019 update on diagnosis, risk stratification, and management.
Shomali W, Gotlib J
Am J Hematol 2019 Oct;94(10):1149-1167. doi: 10.1002/ajh.25617. PMID: 31423623
Hypereosinophilic Syndrome.
Curtis C, Ogbogu P
Clin Rev Allergy Immunol 2016 Apr;50(2):240-51. doi: 10.1007/s12016-015-8506-7. PMID: 26475367
Management of Hypereosinophilic Syndromes.
Roufosse F
Immunol Allergy Clin North Am 2015 Aug;35(3):561-75. doi: 10.1016/j.iac.2015.05.006. PMID: 26209900
FIP1L1/PDGFR alpha-associated systemic mastocytosis.
Yamada Y, Cancelas JA
Int Arch Allergy Immunol 2010;152 Suppl 1(Suppl 1):101-5. Epub 2010 Jun 4 doi: 10.1159/000312134. PMID: 20523072Free PMC Article
KIT D816V-associated systemic mastocytosis with eosinophilia and FIP1L1/PDGFRA-associated chronic eosinophilic leukemia are distinct entities.
Maric I, Robyn J, Metcalfe DD, Fay MP, Carter M, Wilson T, Fu W, Stoddard J, Scott L, Hartsell M, Kirshenbaum A, Akin C, Nutman TB, Noel P, Klion AD
J Allergy Clin Immunol 2007 Sep;120(3):680-7. Epub 2007 Jul 12 doi: 10.1016/j.jaci.2007.05.024. PMID: 17628645

Therapy

S100A8 and S100A9 Promote Apoptosis of Chronic Eosinophilic Leukemia Cells.
Lee JS, Lee NR, Kashif A, Yang SJ, Nam AR, Song IC, Gong SJ, Hong MH, Kim G, Seok PR, Lee MS, Sung KH, Kim IS
Front Immunol 2020;11:1258. Epub 2020 Aug 6 doi: 10.3389/fimmu.2020.01258. PMID: 32903598Free PMC Article
World Health Organization-defined eosinophilic disorders: 2019 update on diagnosis, risk stratification, and management.
Shomali W, Gotlib J
Am J Hematol 2019 Oct;94(10):1149-1167. doi: 10.1002/ajh.25617. PMID: 31423623
Management of Hypereosinophilic Syndromes.
Roufosse F
Immunol Allergy Clin North Am 2015 Aug;35(3):561-75. doi: 10.1016/j.iac.2015.05.006. PMID: 26209900
Eosinophilia in mast cell disease.
Kovalszki A, Weller PF
Immunol Allergy Clin North Am 2014 May;34(2):357-64. Epub 2014 Mar 13 doi: 10.1016/j.iac.2014.01.013. PMID: 24745679Free PMC Article
Murine model of hypereosinophilic syndromes/chronic eosinophilic leukemia.
Yamada Y, Cancelas JA, Rothenberg ME
Int Arch Allergy Immunol 2009;149 Suppl 1:102-7. Epub 2009 Jun 3 doi: 10.1159/000211381. PMID: 19494514

Prognosis

World Health Organization-defined eosinophilic disorders: 2019 update on diagnosis, risk stratification, and management.
Shomali W, Gotlib J
Am J Hematol 2019 Oct;94(10):1149-1167. doi: 10.1002/ajh.25617. PMID: 31423623
Hypereosinophilic Syndrome.
Curtis C, Ogbogu P
Clin Rev Allergy Immunol 2016 Apr;50(2):240-51. doi: 10.1007/s12016-015-8506-7. PMID: 26475367
Generalized Eruptive Histiocytosis Associated With FIP1L1-PDGFRA-Positive Chronic Eosinophilic Leukemia.
Ziegler B, Peitsch WK, Reiter A, Marx A, Goerdt S, Géraud C
JAMA Dermatol 2015 Jul;151(7):766-9. doi: 10.1001/jamadermatol.2015.0154. PMID: 25923837
Mast cells and eosinophils in mastocytosis, chronic eosinophilic leukemia, and non-clonal disorders.
Gotlib J, Akin C
Semin Hematol 2012 Apr;49(2):128-37. doi: 10.1053/j.seminhematol.2012.01.007. PMID: 22449623
KIT D816V-associated systemic mastocytosis with eosinophilia and FIP1L1/PDGFRA-associated chronic eosinophilic leukemia are distinct entities.
Maric I, Robyn J, Metcalfe DD, Fay MP, Carter M, Wilson T, Fu W, Stoddard J, Scott L, Hartsell M, Kirshenbaum A, Akin C, Nutman TB, Noel P, Klion AD
J Allergy Clin Immunol 2007 Sep;120(3):680-7. Epub 2007 Jul 12 doi: 10.1016/j.jaci.2007.05.024. PMID: 17628645

Clinical prediction guides

Venous thrombosis and predictors of relapse in eosinophil-related diseases.
Réau V, Vallée A, Terrier B, Plessier A, Abisror N, Ackermann F, Benainous R, Bohelay G, Chabi-Charvillat ML, Cornec D, Desbois AC, Faguer S, Freymond N, Gaillet A, Hamidou M, Killian M, Le Jeune S, Marchetti A, Meyer G, Osorio-Perez F, Panel K, Rautou PE, Rohmer J, Simon N, Tcherakian C, Vasse M, Zuelgaray E, Lefevre G, Kahn JE, Groh M
Sci Rep 2021 Mar 18;11(1):6388. doi: 10.1038/s41598-021-85852-9. PMID: 33737704Free PMC Article
Generalized Eruptive Histiocytosis Associated With FIP1L1-PDGFRA-Positive Chronic Eosinophilic Leukemia.
Ziegler B, Peitsch WK, Reiter A, Marx A, Goerdt S, Géraud C
JAMA Dermatol 2015 Jul;151(7):766-9. doi: 10.1001/jamadermatol.2015.0154. PMID: 25923837
F604S exchange in FIP1L1-PDGFRA enhances FIP1L1-PDGFRA protein stability via SHP-2 and SRC: a novel mode of kinase inhibitor resistance.
Gorantla SP, Zirlik K, Reiter A, Yu C, Illert AL, Von Bubnoff N, Duyster J
Leukemia 2015 Aug;29(8):1763-70. Epub 2015 Mar 12 doi: 10.1038/leu.2015.70. PMID: 25761934
KIT D816V-associated systemic mastocytosis with eosinophilia and FIP1L1/PDGFRA-associated chronic eosinophilic leukemia are distinct entities.
Maric I, Robyn J, Metcalfe DD, Fay MP, Carter M, Wilson T, Fu W, Stoddard J, Scott L, Hartsell M, Kirshenbaum A, Akin C, Nutman TB, Noel P, Klion AD
J Allergy Clin Immunol 2007 Sep;120(3):680-7. Epub 2007 Jul 12 doi: 10.1016/j.jaci.2007.05.024. PMID: 17628645
The FIP1L1-PDGFRalpha fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management.
Gotlib J, Cools J, Malone JM 3rd, Schrier SL, Gilliland DG, Coutré SE
Blood 2004 Apr 15;103(8):2879-91. Epub 2003 Nov 20 doi: 10.1182/blood-2003-06-1824. PMID: 15070659

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