Classification of chronic myeloid disorders: From Dameshek towards a semi-molecular system

Ayalew Tefferi, Gary Gilliland

Research output: Contribution to journalReview article

31 Scopus citations

Abstract

Hematological malignancies are phenotypically organized into lymphoid and myeloid disorders, although such a distinction might not be precise from the standpoint of lineage clonality. In turn, myeloid malignancies are broadly categorized into either acute myeloid leukemia (AML) or chronic myeloid disorder (CMD), depending on the presence or absence, respectively, of AML-defining cytomorphologic and cytogenetic features. The CMD are traditionally classified by their morphologic appearances into discrete clinicopathologic entities based primarily on subjective technologies. It has now become evident that most CMD represent clonal stem cell processes where the primary oncogenic event has been characterized in certain instances; Bcr/Abl in chronic myeloid leukemia, FIP1L1-PDGFRA or c-kitD816V in systemic mastocytosis, rearrangements of PDGFRB in chronic eosinophilic leukemia, and rearrangements of FGFR1 in stem cell leukemia/lymphoma syndrome. In addition, Bcr/Abl-negative classic myeloproliferative disorders are characterized by recurrent JAK2V617F mutations, whereas other mutations affecting the RAS signaling pathway molecules have been associated with juvenile myelomonocytic leukemia. Such progress is paving the way for a transition from a histologic to a semi-molecular classification system that preserves conventional terminology, while incorporating new information on molecular pathogenesis.

Original languageEnglish (US)
Pages (from-to)365-385
Number of pages21
JournalBest Practice and Research: Clinical Haematology
Volume19
Issue number3
DOIs
StatePublished - Sep 1 2006

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Keywords

  • Bcr/Abl
  • acute myeloid leukemia
  • chronic myeloid disorder

ASJC Scopus subject areas

  • Oncology
  • Clinical Biochemistry

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