Hypereosinophilic syndrome and cyclic oscillations in blood cell counts. A clonal disorder of hematopoiesis originating in a pluripotent stem cell

Luca Malcovati, Roberta La Starza, Serena Merante, Daniela Pietra, Cristina Mecucci, Mario Cazzola

Research output: Contribution to journalArticle

Abstract

We studied a patient with hypereosinophilic syndrome (HES) who had myeloproliferative features, was unresponsive to imatinib mesylate, and showed cyclic oscillations in blood cell counts. No rearrangement in PDGFRA, PDGFRB and ETV6 genes was detected. Clonal analysis of hematopoiesis consistently showed skewed X-chromosome inactivation patterns in both granulocytes and T-lymphocytes, indicating a clonal myeloproliferative disorder originating in a pluripotent stem cell.

Original languageEnglish
Pages (from-to)497-499
Number of pages3
JournalHaematologica
Volume89
Issue number4
Publication statusPublished - Apr 2004

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Hypereosinophilic Syndrome
Platelet-Derived Growth Factor beta Receptor
X Chromosome Inactivation
Myeloproliferative Disorders
Pluripotent Stem Cells
Blood Cell Count
Hematopoiesis
Granulocytes
T-Lymphocytes
Genes
Imatinib Mesylate

Keywords

  • Hematopoiesis
  • Hypereosinophilic syndrome
  • X-chromosome inactivation

ASJC Scopus subject areas

  • Hematology

Cite this

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AU - La Starza, Roberta

AU - Merante, Serena

AU - Pietra, Daniela

AU - Mecucci, Cristina

AU - Cazzola, Mario

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KW - X-chromosome inactivation

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