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Annual Meeting Connective Tissue Oncology Society, Milan, 1998. 43. Mace J, Sybil Biermann J, Sondak V, et al. Response of extraabdominal desmoid tumors to therapy with imatinib mesylate. Cancer. 2002; 95:2373-9. 44. Heinrich MC, McArthur GA, Demetri GD et al. Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumour). J Clin Oncol. 2006; 24:1195-1203. 45. Heinrich MC, Joensuu H, Demetri GD et al. Phase II, openlabel study evaluating the activity of imatinib in treating lifethreatening malignancies known to be

References 1. Demetri GD, von Mehren M, Blanke CD, Van den Abbeele AD, Eisenberg B, Roberts PJ, et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med. 2002; 347:472-80. 2. Schwetz BA. New Treatment for Chronic Myelogenous Leukemia. JAMA. 2001; 286:35. 3. Cohen MH, Williams G, Johnson JR, Duan J, Gobburu J, Rahman A, et al. Approval summary for imatinib mesylate capsules in the treatment of chronic myelogenous leukemia. Clin Cancer Res. 2002; 8: 935-42. 4. Ayoub WS, Geller SA, Tran T, Martin P, Vierling JM

REFERENCES 1. Soverini S, Rosti G, Iacobucci I, et al. Choosing the best second-line tyrosine kinase inhibitor in imatinib-resistant chronic myeloid leukemia patients harboring BCR-ABL kinase domain mutations: How reliable is the IC50? The Oncologist 2011;16: 868-876. 2. Linda E. Johansen. Targeted detection of mutations associated with imatinib-resistance. Strategies to increase sensitivity and specificity [master thesis]. Department of Pharmacology, Institute of Pharmacy, Faculty of Medicine, University of Tromsø, 2005/2006. 3. Shukla I, Suneetha V. Tyrosine

recommendations for harmonizing current methodology for detecting BCR-ABL transcripts and kinase domain mutations and for expressing results. Blood. 2006; 108:28-37. 8. Druker BJ, Guilhot F, O’Brien SG, Gathmann I, Kantarjian H, Gattermann N, et al. Five-year follow-up of patients receiving imatinib for chronic myeloid leukemia. N Engl J Med. 2006; 355:2408-17. 9. Carroll M, Ohno-Jones S, Tamura S, Buchdunger E, Zimmermann J, Lydon NB, et al. CGP 57148, a tyrosine kinase inhibitor, inhibits the growth of cells expressing BCR-ABL, TEL-ABL, and TEL-PDGFR fusion proteins. Blood

References 1. Lichtman LA, Liesveld JL. CML and related disorders, in Williams Hematology, McGraw Hill 8th edition, 1085-1124. 2. Petrov L, Cucuianu A, Bojan A, Urian L. Leucemia granulocitara cronica. In “Hematologie Clinica”. Casa Cartii de Stiinta, Cluj-Napoca, 2009: 45-63 3. Apperley JF. Mechanism of resistance to imatinib in chronic myeloid leukemia. Lancet Oncol, 2007, 8:1018-1029 4. Quintas-Guardama A, Kantarjian H, Cortes J. Mechanism of primary and secondary resistance to imatinib in chronic myeloid leukemia. Cancer Control, 2009, 16: 122-131. 5. Bixby D

other chronic myelogenous leukemia cells. Science. 1984; 225: 72-74. 5. Druker BJ, Tamura S, Buchdunger E, et al. Effects of a selective inhibitor of the Abl tyrosine kinase on the growth of Bcr-Abl positive cells. Nat Med. 1996; 2: 561-566. 6. Goldman JM, Melo JV. Chronic Myeloid leukemia - advances in biology and new approaches to treatment. N Engl J Med. 2003; 349(15): 1451-64. 7. Hughes TP, Kaeda J, Branford S, Rudzi Z, Hochhaus A, Hensly ML, et al. Frequency of major molecular response to imatinib or intereferon alfa plus cytarabine in newly diagnosed chronic


Introduction. Hypereosinophilia (HE) is defined as an absolute eosinophil count >1.5 x 109/L in the peripheral blood on two examinations separated in time by at least one month and/or pathologic confirmation of tissue HE. A hypereosinophilic syndrome (HES) is defined by the association of HE with eosinophil-mediated organ damage and/or dysfunction if other potential causes of them have been excluded.

Case report. We report a case of a 52-year-old woman admitted to Rheumatology Department for diffuse, highly intense (10/10) myalgia, associated with paravertebral, upper and lower limbs muscular edema and stiffness, having the onset of symptoms for about 4 months. During rheumatological evaluation, the patient developed posterior cervical diffuse pain with brachial irradiation associated with severe functional impotence reason for which it was indicated MRI of cervical spine. The blood count showed leucocytosis with hypereosinophilia. No evidence of Raynaud’s phenomenon, cutaneous and muscular sclerosis or visceral involvement. All muscular groups and deep fascia appeared affected on the whole-body MRI and the findings were suggestive for Shulman’s eosinophilic fasciitis or eosinophilic myopahty. All autoimmune, parasitic and allergic causes for myopathy were ruled out. Muscle tissue biopsy revealed high eosinophilic infiltrate predominantly in the deep muscular fascia, in the striated muscles and in the superficial dermis.

The patient received methyl-prednisolone pulse-therapy with a low-maintenance dose of prednisone which showed clinical improvement and normalized peripheral blood eosinophilia. A hematologic evaluation revealed high percentage of bone marrow eosinophil count which harbored FIP1L1-PDGFR alpha mutation and data was suggestive for the diagnosis of myeloproliferative variant of HES syndrome along with Shulman’s eosinophilic fasciitis for which Imatinib treatment was indicated.

Conclusion. The particularity of this case consists in the diagnosis of a myeloproliferative variant of HES with atypical presentation as a Shulman’s eosinophilic fasciitis, a rheumatological condition. Even though initial symptoms were suggestive for a myopathy, it was difficult to establish the positive diagnosis without any obvious paraclinical data. Further investigations such as MRI and muscle biopsy were conclusive for the diagnosis of Shulman’s eosinophilic fasciitis and clinical improvement was observed after corticosteroid treatment. Considering that after 4 months of evolution of the disease the patient did not present any atrophic lesions of the affected muscles and skin characteristic for Shulman’s eosinophilic fasciitis the patient was referred to Hematology Department with the diagnosis of HES. Advanced exams established the hematological condition as primary FIP1L1-PDGFR positive HES which could benefit from tyrosine-kinase inhibitors treatment (Imatinib) known to induce in this situation improved clinical and paraclinical status.

, and some were approved for soft tissue sarcoma. The fi rst approved was Imatinib, as a treatment for gastrointestinal stromal tumors (GISTs). Following Imatinib, other tyrosine kinase inhibitors (TKIs) received the approval for GISTs such as Sunitinib and Regorafenib, and Pazopanib for non-GIST soft tissue sarcomas. In 2016, FDA approved the fi rst monoclonal antibody that targets platelet-derived growth factor receptor (PDGFR)-α, Olaratumab. The new treatment demonstrates an overall survival advantage. In this review, we aimed to summarize the results from

? Blood. 2011;117:755-763. 5. Belli C, Alú MF, Alfonso G, Bianchini M, Larripa I. Novel variant Ph translocation t(9;22;11)(q34;q11.2;p15)inv(9)(p13q34) in chronic myeloid leukemia involving a one-step mechanism. Cytogenet Genome Res. 2011;132(4):304-8. Epub 2011 Jan 6. 6. Jabbour E, Cortes J, Kantarjian H. Optimal First-Line Treatment of Chronic Myeloid Leukemia: How to Use Imatinib and What Role for Newer Drugs? Oncology. 2007;21(6):653-62. 7. Litzow MR. Imatinib resistance: obstacles and opportunities. Arch Pathol Lab Med. 2006;130:669-679. 8. Deininger MW


The coexistence of t(9;22) and inv(16) has been described in a very limited number of cases of CML, de novo or therapy-related AML. We report a patient with CML who presented both inversion of chromosome 16 and Philadelphia chromosome and evolved towards the blast phase under treatment with Imatinib. Laboratory diagnosis and monitoring was made by flow cytometry, conventional cytogenetics and molecular genetics techniques. The inv(16), detected by karyotyping in the Philadelphia chromosome positive clone at the moment of the blast transformation, was retrospectively assessed by means of real-time PCR, and was proved to have been present since diagnosis. The bone marrow biopsy performed in the blast phase of CML confirmed the presence of blasts belonging to the myeloid lineage, with indications of monocytic differentiation, frequently associated with inv(16). Moreover, the case also associated a F359V tyrosine kinase domain mutation, resulting in intermediate resistance to Imatinib and Nilotinib, which imposed therapy-switch to Dasatinib. In our case the evolution was progressive, followed by death due to lack of response to tyrosine kinase inhibitors, 18 months after diagnosis. The coexistence of t(9;22) and inv(16) in CML seems to be associated with an aggressive clinical evolution and resistance to tyrosine kinase inhibitor therapy. Due to the very small number of cases described in literature, therapeutic decisions are still difficult for patients displaying these abnormalities