Successful Treatment For Chronic Eosinophilic Leukemia (CEL) With Imatinib Mesylate

  • Rayane da Silva Souza
  • Nathalia de Alencar Cunha Tavares
  • Martina Bragante Fernandes Pimenta
  • Marcelle Bragante Fernandes Pimenta
  • Matheus Cartaxo Eloy Fialho
  • Ary Serrano Santos
  • Alexandre Rolim da Paz
  • Flávia Cristina Fernandes Pimenta Federal University of Paraíba


We report a case of a patient with Chronic Eosinophilic Leukemia (CEL) with mutation in alfa PDGFR gene exhibiting a satisfactory response to treatment with imatinib mesylate. A 25-year-old man presented in a hematology service with a persistent cough and hemogram alterations. His blood count showed a hemoglobin level of 12.5 g/dL and a white blood cell count of 94,030/mm3, eosinophils were 68% of all cells. Bone marrow aspiration and biopsy showed hypercellularity with marked eosinophilia (77%) and erythroid differentiation series was hypocellular with normoblast maturation. The immunohistochemically of the bone biopsy was positive for myeloperoxidase and negative for CD34/CD99, consistent with CEL. Fluorescence in situ hybridization (FISH) for the beta-fraction of platelet-derived growth factor (PDGFRβ) and Philadelphia chromosome (Ph 1) were negative and the alfa PDGFR (Platelet-Derived Growth Factor) was positive and showed heterozygosis in c.2531T>C on 18 Exon and homozygous in C.2562+1G>A at the region of the splicing site at the 18 intron. Treatment was initiated and maintained by administering 400mg/day imatinib mesylate. Laboratory findings returned to normal ranges, with clinical improvement and a hematological response observed after the second month of therapy. Currently, the patient’s blood count shows the white blood cell count (5,400 total leukocytes), eosinophils (8.6/mm3), hemoglobin (15.5 g/dl), hematocrit (45.4%) and platelets (298,000/mm3) within normal ranges. The mutation search was negative in in peripheral blood one year after the initial treatment. Our work corroborates other studies on the efficacy of imatinib mesylate in the treatment of patients with CSF PDGFR alpha positive. We emphasize the importance of molecular studies, considering its relevance for the correct staging of the disease. Since CEL is a rare disease, it is important to define its etiology and anticipate its treatment, thus minimizing the damage induced by the disease.

Author Biographies

Rayane da Silva Souza

Department of Medicine, Federal University of Paraíba, João Pessoa, Paraíba, Brazil.

Nathalia de Alencar Cunha Tavares

Lauro Wanderley University Hospital - Federal University of Paraíba, João Pessoa, Paraíba, Brazil.

Martina Bragante Fernandes Pimenta

Department of Medicine, Federal University of Paraíba, João Pessoa, Paraíba, Brazil

Marcelle Bragante Fernandes Pimenta

Department of Medicine, Nova Esperança College, João Pessoa, Paraíba, Brazil

Matheus Cartaxo Eloy Fialho

Department of Medicine, Federal University of Paraíba, João Pessoa, Paraíba, Brazil.

Ary Serrano Santos

Lauro Wanderley University Hospital - Federal University of Paraíba, João Pessoa, Paraíba, Brazil

Alexandre Rolim da Paz

Department of Medicine, Federal University of Paraíba, João Pessoa, Paraíba, Brazil.

Lauro Wanderley University Hospital - Federal University of Paraíba, João Pessoa, Paraíba, Brazil


1. Sheikh J, Weller PF: Advances in diagnosis and treatment of
eosinophilia. Curr Opin in Hematol. 2009 Jan; 16(1):3-8.
2. Spry C: Eosinophilia in Addison's Disease. Yale J Biol and Med.
1976 Sep; 49(4): 411-13.
3. Jennette JC, Falk RJ, Bacon PA, N. Basu, M. C. Cid, F. Ferrario et
al: 2012 Revised international Chapel Hill consensus conference
nomenclature of vasculitides. Arthritis Rheum. 2013 Jan;
4. Ackerman SJ, Bochner BS: Mechanisms of eosinophilia in the
pathogenesis of hypereosinophilic disorders. Immunol Allergy
Clin of North Am. 2007 Aug; 27(3):357-75.
5. Valent P, Klion AD, Horny HP, Roufosse F, Gotlib J, Weller PF, et al:
Contemporary consensus proposal on criteria and classification
of eosinophilic disorders and related syndromes. J Allergy Clin
Immunol. 2012 Sep; 130(3):607-12.e9
6. Bain BJ, Gilliland DG, Horny H-P, et al. Chronic eosinophilic
leukaemia, not otherwise specified. In: Swerdlow S, Harris NL,
Stein H, Jaffe ES, Theile J, Vardiman JW, editors. World Health
Organization Classification of Tumours. Pathology and Genetics
of Tumours of Haematopoietic and Lymphoid Tissues. Lyon,
France, IARC Press. 2008. p 51-3.
7. Gotlib, J. World Health Organization-defined eosinophilic
disorders: 2015 update on diagnosis, risk stratification, and
management. American Journal of Hematology. 2015 Nov;
90(11) :1077- 89
8. Gotlib J, Cools J. Five years since the discovery of FIP1L1-PDGFRA:
what we have learned about the fusion and other molecularly
defined eosinophilias. Leukemia. 2008 Nov; 22(11):1999-2010.
9. Valent P: Pathogenesis, classification, and therapy of eosinophilia
and eosinophil disorders. Blood Reviews. 2009 Jul; 23(4):157-65.
10. Cool J, Stover EH, Włodarska I Marynen P, Gilliland DG: The
FIP1L1- PDGFRa kinase in hypereosinophilic syndrome and
chronic eosinophilic leukemia. Current Opinion in Hematology
2004 Jan; 11(1):51-7.
11. Weller PF, Bubley J: The Idiopathic hypereosinophilic syndrome.
Blood 1994 May 15; 83(10):2759-79.
12. Helbig G, Hus M, Francuz T, Dziaczkowska-Suszek J, Soja A,
Kyrcz-Krzemień S: Characteristics and clinical outcome of
patients with hypereosinophilia of undetermined significance.
Med Oncol. 2014 Jan; 31(1):815.
13. Arruda MMAS, Sandes AF, Borges NM, Chauffaille MLLF:
Chronic eosinophilic leukemia with expression of FIP1L1-PDGFR
rearrangement - case report and literature review. J Hematol
2010 May; 32(2):177-80.
14. Akuthota P, Weller PF: Eosinophils and disease pathogenesis.
Seminars in Hematology 2012 Apr; 49(2): 113-119.
15. Shin SY1, Jung CW2, Choi DC2, Lee BJ2, Kim HJ1, Kim
SH1.: Chronic eosinophilic leukemia with a FIP1L1-PDGRFA
rearrangement: Two case reports and a review of Korean cases.
Blood Reviews. 2015 Mar; 50(1):58-61.
16. Cortes J, Hochhaus A, Hughes T, Kantarjian, H: Front-line and
salvage therapies with tyrosine kinase inhibitors and other
treatments in chronic myeloid leukemia. Journal of Clinical
Oncology. 2011 Feb 10; 29(5): 524-531.
17. Schaller JL, Burkland GA. Case report: rapid and complete
control of idiopathic hypereosinophilia with imatinib mesylate.
Medscape General Medicine. 2001 Sep 7; 3(5):9.
18. Brito-Babapulle F. The eosinophilias, including the idiopathic
hypereosinophilic syndrome. British Journal of Haematology
2003 Apr; 121(2):203-23.
19. Gleich GJ, Leiferman KM, Pardanani A, Tefferi A, Butterfield
JH.. Treatment of hypereosinophilic syndrome with imatinib
mesilate. Lancet. 2002 May 4; 359(9317):1577-8.
20. Helbig G, Hus M, Hałasz M, Dudziński M, Więcławek A,
Stachowicz M, et al. Imatinib mesylate may induce long-term
clinical response in FIP1L1-PDGFRα-negative hypereosinophilic
syndrome. Medical Oncology. 2012 Jun; 29(2):1073-6.
21. Iurlo A, Fracchiolla NS, Ferla V, Cassin R, Gottardi E, Beghini
A, et al. Successful Treatment With Imatinib in a Patient With
Chronic Eosinophilic Leukemia Not Otherwise Specified. Journal
Of Clinical Oncology. 2014 Apr 1; 32(10):e37-9.
22. Arber, Daniel A, Orazi, Attilio, Hasserjian, Robert, Thiele, J¨urgen,
Borowitz, Michael J, Le Beau, Michelle M. The 2016 revision
to the World Health Organization classification of myeloid
neoplasms and acute leukemia (The Updated Who Classification
of Hematological Malignancies). BLOOD, 2016 19 May; 127(20):
How to Cite
SOUZA, Rayane da Silva et al. Successful Treatment For Chronic Eosinophilic Leukemia (CEL) With Imatinib Mesylate. International Archives of Medicine, [S.l.], v. 10, dec. 2017. ISSN 1755-7682. Available at: <>. Date accessed: 13 dec. 2019. doi: