Isolated isochromosome 17q: A distinct type of mixed myeloproliferative disorder/myelodysplastic syndrome with an aggressive clinical course

R. F. McClure, G. W. Dewald, J. D. Hoyer, Curtis A. Hanson

Research output: Contribution to journalArticle

53 Scopus citations

Abstract

A clinicopathologic study was performed on 15 patients with haematological malignancies in which isochromosome 17q [i(17q)] was the sole structural chromosome abnormality identified in bone marrow. The data indicated that an isolated i(17q) is associated with a distinct type of mixed chronic myeloproliferative/myelodysplastic disorder with an aggressive clinical course. The patients ranged in age from 37 to 83 years (median 60) with a M:F ratio of 3:1. All cases were chronic myeloid disorders with mixed proliferative and dysplastic features, making classification difficult. 11 patients tested for BCR/ABL gene fusion were normal. A low bone marrow blast count (<5%) at presentation was a typical finding. All cases had severe myeloid dysplasia which included non-segmented neutrophils and an increase in the monocyte/macrophage lineage. Fluorescence in situ hybridization (FISH) analysis of one case showed the i(17q) to involve all myeloid lineages, but not the lymphocytes. For cases with complete follow-up (n = 11) the median survival was 2.5 years (range 0.83-5.25) and 64% progressed to AML prior to death. The following features were identified which defined the haematological disorder associated with an isolated i(17q): (1) adult patient, (2) chronic myeloid disorder with clonal involvement of all myeloid lineages, (3) mixed chronic myeloproliferative/myelodysplastic features, (4) severe hyposegmentation of neutrophil nuclei, (5) prominence of the monocyte/macrophage lineage, (6) high risk for progression to AML, and (7) median survival of 2-5 years.

Original languageEnglish (US)
Pages (from-to)445-454
Number of pages10
JournalBritish journal of haematology
Volume106
Issue number2
DOIs
StatePublished - Oct 5 1999

Keywords

  • Chronic myeloproliferative disorder
  • Isochromosome 17q
  • Myelodysplastic syndrome
  • Pseudo-Pelger-Huet neutrophils

ASJC Scopus subject areas

  • Hematology

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