Hypereosinophilic syndrome with evolution to myeloproliferative disorder: Temporal relationship to loss of Y chromosome and c-N-ras activation

S. W. Needleman, S. M. Mane, J. C. Gutheil, V. Kapil, M. R. Heyman, J. R. Testa

Research output: Contribution to journalArticlepeer-review

23 Scopus citations

Abstract

A case of hypereosinophilic syndrome is presented in which the patient was serially observed for 4 years. Transformation to a disorder resembling chronic myeloid leukemic (CML) occurred 36 months after diagnosis; at 42 months, blastic transformation and marrow failure ensued, leading to death. Marrow examination for histopathologic, cytogenetic, and molecular biologic analyses were performed during the eosinophilic, myeloproliferative, and blastic stages. These demonstrated ras activation by virtue of a codon 12 G to C transversion mutation, predicting for substitution of glycine by alanine; in addition, we observed Y chromosome loss late in the natural history of this illness, suggesting that these genetic lesions can play a role in the profound loss of myeloid differentiation characteristic of the accelerated phase commonly observed in myeloproliferative syndromes.

Original languageEnglish
Pages (from-to)149-155
Number of pages7
JournalHematologic Pathology
Volume4
Issue number3
StatePublished - 1990

Keywords

  • Adult
  • Base Sequence
  • DNA, Neoplasm/isolation & purification
  • DNA, Single-Stranded/chemical synthesis
  • Eosinophilia/complications
  • Gene Expression Regulation, Neoplastic/physiology
  • Genes, ras/genetics
  • Humans
  • Karyotyping
  • Leukemia, Myeloid/etiology
  • Longitudinal Studies
  • Male
  • Molecular Sequence Data
  • Myeloproliferative Disorders/etiology
  • Polymerase Chain Reaction
  • Sex Chromosome Aberrations/genetics
  • Syndrome
  • Time Factors
  • Y Chromosome

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