Clonal Hypereosinophilia with ETV6 Rearrangement Evolving to T-Cell Lymphoblastic Lymphoma: A Case Report and Review of the Literature.

Case Rep Hematol

Hematology Department, Instituto Português de Oncologia de Lisboa, Francisco Gentil, Rua Professor Lima Basto, 1099-213 Lisbon, Portugal.

Published: May 2013

AI Article Synopsis

  • A case study highlights a patient with hypereosinophilia that progressed to T-cell lymphoblastic lymphoma, treated successfully with chemotherapy.
  • Recurrence of hypereosinophilia occurred five months later, coinciding with myeloblastic bone marrow involvement but no return of lymphoma symptoms.
  • Genetic analysis indicated a complex chromosomal translocation and ETV6 gene rearrangement, suggesting a clonal nature of the eosinophilic disorder, similar to other conditions linked to FGFR1 rearrangements.

Article Abstract

Hypereosinophilia, either clonal or reactive, has been described in association with multiple hematological malignancies. We describe a case of a patient presenting with hypereosinophilia that evolved into T-cell lymphoblastic lymphoma. Complete remission was achieved with chemotherapy; however, hypereosinophilia recurred 5 months later in association with myeloblastic bone marrow infiltration and without evidence of lymphoblastic lymphoma relapse. Cytogenetic analysis of the bone marrow showed a complex translocation involving chromosomes 7, 12, and 16. A rearrangement of ETV6 gene (12p13) was demonstrated by FISH studies, thus confirming the clonality of this population. The association of lymphoblastic lymphoma, eosinophilia, and myeloid hyperplasia has been described in disorders with FGFR1 rearrangements. We hypothesize that other clonal eosinophilic disorders lacking this rearrangement could behave in a similar fashion through different pathogenic mechanisms.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3654631PMC
http://dx.doi.org/10.1155/2013/652745DOI Listing

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