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Eur J Haematol. 2009 Apr;82(4):255-9. doi: 10.1111/j.1600-0609.2009.01216.x. Epub 2008 Feb 10.

Karyotype complements the International Prognostic Scoring System for primary myelofibrosis.

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  • 1Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA.

Abstract

OBJECTIVES:

The International Prognostic Scoring System (IPSS) for primary myelofibrosis (PMF) is based on five independent predictors of inferior survival: age >65 yr, hemoglobin <10 g/dL, leukocyte count >25 x 10(9)/L, circulating blasts > or =1%, and presence of constitutional symptoms. The presence of 0, 1, 2, and > or =3 adverse factors defines low, intermediate-1, intermediate-2, and high risk disease, respectively. We examined the additional prognostic relevance of karyotype.

METHODS:

World Health Organization criteria were used for PMF diagnosis. Only patients with bone marrow cytogenetic studies at the time or within 1 yr of diagnosis and a minimum of 20 evaluable metaphases were considered. Cytogenetic findings were categorized as 'normal' vs. 'abnormal' or 'favorable' (normal or with sole abnormalities of 13q- or 20q-) vs. 'unfavorable' (all other abnormalities).

RESULTS:

A total of 109 patients were studied (median age 63 yr). Numbers of patients in the above-listed four IPSS risk groups were 26, 31, 28, and 24, respectively. Cytogenetic results were abnormal in 33% of the patients and unfavorable in 21%. At a median follow-up of 35 months, 45 (41%) deaths were recorded. 'Unfavorable' (P = 0.008) but not 'abnormal' (P = 0.19) karyotype predicted shortened survival and its significance remained on multivariable analysis that included the IPSS or other prognostic tools as covariates. JAK2V617F, detected in 63 (58%) patients, was inconsequential to survival.

CONCLUSIONS:

In PMF, specific cytogenetic abnormalities and not the mere presence of an abnormal karyotype provide important prognostic information that is not accounted for by the IPSS or other established risk factors.

PMID:
19215287
[PubMed - indexed for MEDLINE]
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