A chronic eosinophilic leukemia patient presenting with blurred vision / Bulanik gormeyle basvuran kronik eozinofilik losemi hastasi.
Platelet-derived growth factor
|Publication:||Name: Turkish Journal of Hematology Publisher: Aves Yayincilik Audience: Academic Format: Magazine/Journal Subject: Health Copyright: COPYRIGHT 2011 Aves Yayincilik ISSN: 1300-7777|
|Issue:||Date: Sept, 2011 Source Volume: 28 Source Issue: 3|
Chronic eosinophilic leukemia (CEL), a rare entity, is classified
by the WHO as 2 groups of patients with neoplastic eosinophils: those
that have a myeloid or lymphoid neoplasm with eosinophilia, and
abnormalities in PDGFRA, PDGFRB, or FGFR1 genes, and those integrated in
a subchapter as MPN that have chronic eosinophilic leukemia, not
otherwise specified (1-3). Hepatosplenomegaly, eosinophilia with
systemic disturbances, anemia, thrombocytopenia, and in particular
cardiac involvement are major components of the disease (4).
A 32-year-old male that had previously been healthy presented to our clinic with fatigue, blurred vision, non-productive cough, and sore throat that began 10 d earlier. Physical examination showed splenomegaly 10 cm below the costal margin, and segmentation and dilatation of capillaries around the optic disc. His hemogram results were as follows: Hg: 9.2 g/dL, WBC: 50,000/[micro]L, Plt count: 98,000/[micro]L, eosinophils: 21,100/[micro]L. Peripheral blood smear showed 62% eosinophils. Bone marrow aspiration showed <50% myeloid cells that were eosinophils and [less than or equal to]3% blasts.
Fluorescent in situ hybridization analysis of t(9; 22) was negative. Tests for BCR-ABL and FIP1L1-PDGFRA rearrangement with real time PCR were performed. Parasite tests for eosinophilia were negative. Hypotensive attacks with dynamic electro-cardiographic changes were observed and methylprednisolone 1 mg/kg/d was initiated following observation of pericardial effusion. The effusion resolved, but despite steroid therapy his eosinophil count did not change as expected, and red blood cell and thrombocyte transfusions were required during follow-up. BCR-ABL rearrangement was negative and FIPL1-PDGFRA rearrangement of RNA was observed; therefore, imatinib 100 mg/d was initiated and steroid therapy was immediately withdrawn. His clinical condition quickly improved, eosinophilia disappeared, vision improved, and he was transfusion free. The patient was discharged in good health and during his last outpatient follow-up his Hg was 12.3 g/dL, WBC was 5600/[micro]L, Plt count was 148,000/[micrp]L, and eosinophils was 0.4%.
CEL, once a disease with a poor prognosis, is now a manageable disease associated with a good quality of life using tyrosine kinase inhibitors. The FIP1L1-PDGFRA fusion gene was reported to be 50-fold more sensitive to the tyrosine kinase inhibitor imatinib (5) than BCR-ABL, and as such lowdose (100 mg/d) imatinib rapidly achieves hematologic and molecular response. The presented case was transfusion-dependent and had vision loss attributed to eosinophilia; however, following initiation of imatinib therapy his clinical and hematologic condition quickly improved.
We had obtained written informed consent from the patient.
Conflict of interest statement
The authors of this paper have no conflicts of interest, including specific financial interests, relationships, and/or affiliations relevant to the subject matter or materials included.
(1.) Valent P. Pathogenesis, classification and therapy of eosinophilia and eosinophil disorders. Blood Reviews 2009;23:157-65.
(2.) Bain B, Gilliland DG, Vardiman JW, Horny HP Chronic eosinophilic leukaemia, not otherwise specified. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Piled SA, Stein H, Thiele J, Vardiman JW, editors. WHO classification of tumours of haematopoietic and lymphoid tissues, vol. 2. Geneva (Switzerland) and Albany (NY, USA): WHO Press; 2008;51-3.
(3.) Bain B, Gilliland DG, Horny HP, Vardiman JW. Myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB, orFGFR1. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, editors. WHO classification of tumours of haematopoietic and lymphoid tissues, vol. 2. Geneva (Switzerland) and Albany (NY, USA): WHO Press; 2008;68-73.
(4.) Sheikh J, Weller FW. Advances in diagnosis and treatment of eosinophilia. Current Opinion in Hematology 2009;16:3-8.
(5.) Cools J, DeAngelo DJ, Gotlib J, Stover EH, Legare RD, Cortes J, Kutok J, Clark J, Galinsky I, Griffin JD, Cross NC, Tefferi A, Malone J, Alam R, Schrier SL, Schmid J, Rose M, Vandenberghe P, Verhoef G, Boogaerts M, Wlodarska I, Kantarjian H, Marynen P, Coutre SE, Stone R, Gilliland DG. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med 2003;348:1201-14.
Demircan Ozbalci (1), Clku Ergene (1), Harun Yazgan (2)
(1) Department of Hematology, Faculty of Medicine, Celal Bayar University, Manisa, Turkey
(2) Department of Internal Medicine, Faculty of Medicine, Celal Bayar University, Manisa, Turkey
Address for Correspondence: Dr. Demircan Ozbalci, Department of Hematology Faculty of Medicine, Celal Bayar University, Manisa, Turkey Phone: +90 236 233 07 25 E-mail: firstname.lastname@example.org
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