Myeloid neoplasia with eosinophilia with PDGFRA reordention with pulmonary and cutaneous manifestations : about a case.
Neoplasia mieloide con eosinofilia con el reordenamieno PDGFRA con manifestaciones pulmonares y cutáneas : a propósito de un caso.
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Hypereosinophilia is present in a group of disorders with excessive production of eosinophils capable of causing damage to organs and systems. The genetic and molecular study of these entities has allowed the classification of a group of myeloid or lymphoid neoplasms of variable clinical evolution. In myeloid neoplasm with eosinophilia, lung involvement is relatively frequent, however the presentation with acute respiratory distress syndrome (ARDS) is very unusual. In this article we present the case of a male patient with rapid onset of respiratory symptoms, hepatosplenomegaly, anemia, leukosytosis with eosinophilia, and detection of FIP1L1-PDGFRA rearrangement that established a diagnosis under WHO criteria of mieloid neoplasia with eosinophilia and PDGFRA rearrangement. Treatment was started with imatinib achieving rapid resolution of hematological and respiratory symptoms.
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- Reiter A, Gotlib J. Myeloid neoplasms with eosinophilia. Blood. 2017;129(6):704-14.
- Gotlib J. World Health Organization-defined eosinophilic disorders: 2014 update on diagnosis, risk stratification, and management. Am J Hematol. 2014;89(3):325-37.
- Dulohery MM, Pate RR, Schneider F, Ryu JH. Lung involvement in hypereosinophilic syndromes. Respir Med. 2011;105(1):114-21.
- Winn RE, Kollef MH, Meyer JI. Pulmonary involvement in the hypereosinophilic syndrome. Chest. 1994;105(3):656-60.
- Jain A, Malhotra P, Suri V, Agarwal A, Bal A, Varma S. A young man with acute respiratory distress syndrome: eosinophilia is not always “benign”. Blood Res. 2017;52(4):329-32.
- Swerdlow SH, Campo E, Harris NL, Jaffe E, et al. World Heath Organization classification of tumours of heamatopoietic and lymphoid tissues. 4a ed. Lyon: IARC; 2017.
- Cools J, DeAngelo DJ, Gotlib J, Stover EH, Legare RD, Cortes J, et al. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med. 2003;348(13):1201-14.
- Vanderberghe P, Wlodarska I, Michaux L, Zachée P, Boogaerts M, Vanstraelen D, et al. Clinical and molecular features of FIP1L1-PDGFRA (+) chronic eosinophilic leukemias. Leukemia. 2004;18(4):734-42.
- Lierman E, Folens C, Stover EH, Mentens N, Van Miegroet H, Scheers W, et al. Sorafenibis a potent inhibitor of FIP1L1-PDGFRalpha and the imatinib-resistant FIP1L1-PDGFRalpha T674I mutant. Blood. 2006;108(4):1374-6.
- Cools J, Stover EH, Boulton CL, Gotlib J, Legare RD, Amaral SM, et al. PCK412 overcomes resistance to imatinib in a murine model of FIP1L1-PDGFRα-induced myeloproliferative disease. Cancer Cell. 2003;3(5):459-69.
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