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November 3, 2016Pediatric Blood & Cancer5 citations

Pediatric 8p11 eosinophilic myeloproliferative syndrome (EMS): A case report and review of the literature

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JSJay F. SarthyNRNaresh ReddivallaMRMohamed Radhi

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Abstract

The 8p11 eosinophilic myeloproliferative syndrome (EMS) is an aggressive neoplasm driven by translocation of the fibroblast growth factor receptor 1 and often transforms to leukemias and lymphomas that are refractory to treatment. The first case was identified in 1983, and to date over 70 cases have been reported in the literature. Despite those reports, no consensus exists on management of this condition, and inconsistency in treatment regimens is even more pronounced in the pediatric literature. We report a case of a male infant with the 8p11 EMS, review the published pediatric experience with EMS, and discuss treatment strategies for this enigmatic hematological disorder.

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Sarthy et al. (2016) studied this question.

synapsesocial.com/papers/6a23c84c003b3cd7fea6114fhttps://doi.org/10.1002/pbc.26310
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1A Case of 8p11 Myeloproliferative Syndrome with BCR-FGFR1 Gene Fusion Presenting with Trilineage Acute Leukemia/Lymphoma, Successfully Treated by Cord Blood Transplantation2012 · 31 citations
  2. 2A young child with acquired t(8;9)(p11;q34): additional proof that 8p11 is involved in mixed myeloid/T lymphoid malignancies.1996 · 23 citations
  3. 3Chronic myelomonocytic leukaemia with t(8;9)(p11;q34) in childhood: an example of the 8p11 myeloproliferative disorder?1996 · 33 citations
  4. 4Philadelphia chromosome-negative chronic myelogenous leukemia in a child with t(8;9)(p11 or 12;q34).1983 · 34 citations
  5. 5T-Cell Lymphoblastic Lymphoma With Eosinophilia Associated With Subsequent Myeloid Malignancy1992 · 121 citations