Mast cell disease followed by leukemia with clonal evolution

Jerry P. Lewis, Jeanna L Welborn, Frederick J Meyers, Norman B. Levy, Thomas Roschak

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

A 29-yr old woman developed urticaria pigmentosa which subsequently progressed through systemic mastocytosis to Philadelphia chromosome negative (Ph neg) chronic myelogenous leukemia (CML) with t(8;17). Further cytogenetic evolution occurred at the time of transformation to the aggressive phase of the disease. Unlike Ph-positive CML, chromosome number 9 was not involved, nor was the breakpoint cluster region located at band 22q11. This clearly separates this case from other Ph-negative CML patients who do have involvement of 9q34[1] or the breakpoint cluster region [2, 3]. Since this is the first case of its type to be reported with cytogenetic abnormalities, the clinical relevance of the unique chromosomal rearrangement t(8;17)(pll;q25) in the setting of systemic mastocytosis is unclear. Additional cases need to be reported to determine if this genetic rearrangement is a nonrandom marker of leukemia evolving in a setting of malignant mast cell disease.

Original languageEnglish (US)
Pages (from-to)769-773
Number of pages5
JournalLeukemia Research
Volume11
Issue number9
DOIs
StatePublished - 1987

Fingerprint

Clonal Evolution
Mastocytosis
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Systemic Mastocytosis
Leukemia
Urticaria Pigmentosa
Philadelphia Chromosome
Chromosomes, Human, Pair 9
Cytogenetics
Chromosome Aberrations

Keywords

  • chromosome abnormality
  • Mast cell leukemia

ASJC Scopus subject areas

  • Cancer Research
  • Hematology
  • Oncology

Cite this

Mast cell disease followed by leukemia with clonal evolution. / Lewis, Jerry P.; Welborn, Jeanna L; Meyers, Frederick J; Levy, Norman B.; Roschak, Thomas.

In: Leukemia Research, Vol. 11, No. 9, 1987, p. 769-773.

Research output: Contribution to journalArticle

Lewis, Jerry P. ; Welborn, Jeanna L ; Meyers, Frederick J ; Levy, Norman B. ; Roschak, Thomas. / Mast cell disease followed by leukemia with clonal evolution. In: Leukemia Research. 1987 ; Vol. 11, No. 9. pp. 769-773.
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