A retrospective analysis of myelodysplastic syndromes with thrombocytosis: reclassification of the cases by WHO proposals

Cabello, A; Collado, Rosa; Ruiz, María Ángeles; Martı́nez, Jesús; Navarro, Isabel; Ferrer, Rafael Alemany; Sosa, Amparo Miguel; Carbonell, Félix

doi:10.1016/j.leukres.2004.07.014

Cited by 27 publications

(8 citation statements)

References 28 publications

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“…In addition to their clinical usefulness, such models might overcome consequences of diagnostic imprecision and provide context for the design and interpretation of clinical trials. In this regard, despite our concerted effort to exclude occult cases of 'prefibrotic' MMM 20 and MDS with thrombocythemia, 26 we recognize the possibility that the inadvertent inclusion of such cases in our high-risk patient categories might have influenced their outcome. Nevertheless, we would argue that our diagnostic procedures are comparable to those of the practice in the wider community and the particular concern should not undermine the overall practical value of the proposed prognostic models.…”

Section: Discussionmentioning

confidence: 97%

Risk stratification for survival and leukemic transformation in essential thrombocythemia: a single institutional study of 605 patients

et al. 2006

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Unlike the case with thrombosis, prognostic models for survival and leukemic transformation (LT) in essential thrombocythemia (ET) are not available. Among 605 patients with ET seen at our institution and followed for a median of 84 months, 155 died and LT was documented in 20 patients (3.3%). In a multivariable analysis, hemoglobin level below normal (females o120 g/l; males o135 g/l) was identified as an independent risk factor for both inferior survival and LT. Additional risk factors for survival included age X60 years, leukocyte count X15 Â 10 9 /l, smoking, diabetes mellitus and thrombosis. For LT, platelet count X1000 Â 10 9 /l but not cytoreductive therapy was flagged as an additional independent risk factor. In fact, four of the 20 patients (20%) with LT were untreated previously. We used the above information to construct prognostic models that effectively discriminated among low-, intermediate-and high-risk groups with respective median survivals of 278, 200 and 111 months (Po0.0001), and LT rates of 0.4, 4.8 and 6.5% (P ¼ 0.0009) respectively. Presence of JAK2V617F did not impact either survival or LT and mutational frequency was similar among the different risk groups.

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Section: Discussionmentioning

confidence: 97%

Risk stratification for survival and leukemic transformation in essential thrombocythemia: a single institutional study of 605 patients

et al. 2006

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“…Some cases of MPD show a pathophysiologic overlap with MDS [21], [22], [31], and in some instances, the JAK2 mutation is present. A significant clinical heterogeneity, ranging from common chronic course to less frequent evolution to AML [13], [16]–[18], exists among classical MPD patients with the JAK2 V617F mutation, and is even more diverse in patients with MDS/MPD overlap.…”

Section: Discussionmentioning

confidence: 99%

SNP Array Karyotyping Allows for the Detection of Uniparental Disomy and Cryptic Chromosomal Abnormalities in MDS/MPD-U and MPD

et al. 2007

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We applied single nucleotide polymorphism arrays (SNP-A) to study karyotypic abnormalities in patients with atypical myeloproliferative syndromes (MPD), including myeloproliferative/myelodysplastic syndrome overlap both positive and negative for the JAK2 V617F mutation and secondary acute myeloid leukemia (AML). In typical MPD cases (N = 8), which served as a control group, those with a homozygous V617F mutation showed clear uniparental disomy (UPD) of 9p using SNP-A. Consistent with possible genomic instability, in 19/30 MDS/MPD-U patients, we found additional lesions not identified by metaphase cytogenetics. In addition to UPD9p, we also have detected UPD affecting other chromosomes, including 1 (2/30), 11 (4/30), 12 (1/30) and 22 (1/30). Transformation to AML was observed in 8/30 patients. In 5 V617F+ patients who progressed to AML, we show that SNP-A can allow for the detection of two modes of transformation: leukemic blasts evolving from either a wild-type jak2 precursor carrying other acquired chromosomal defects, or from a V617F+ mutant progenitor characterized by UPD9p. SNP-A-based detection of cryptic lesions in MDS/MPD-U may help explain the clinical heterogeneity of this disorder.

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“…3 'MDS/MPD, unclassifiable' includes the WHO provisional entity of 'refractory anemia with ringed sideroblasts associated with marked thrombocytosis (RARS-T)'; however, the use of the term RARS-T should be restricted to patients who display both dyserythropoiesis (in addition to ringed sideroblasts) and megakaryocytes similar to those in ET, PV or PMF. 22,23 The 2008 WHO classification of myeloproliferative neoplasms…”

Section: Introductionmentioning

confidence: 99%

Classification and diagnosis of myeloproliferative neoplasms: The 2008 World Health Organization criteria and point-of-care diagnostic algorithms

2007

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A retrospective analysis of myelodysplastic syndromes with thrombocytosis: reclassification of the cases by WHO proposals

Cited by 27 publications

References 28 publications

Risk stratification for survival and leukemic transformation in essential thrombocythemia: a single institutional study of 605 patients

Risk stratification for survival and leukemic transformation in essential thrombocythemia: a single institutional study of 605 patients

SNP Array Karyotyping Allows for the Detection of Uniparental Disomy and Cryptic Chromosomal Abnormalities in MDS/MPD-U and MPD

Classification and diagnosis of myeloproliferative neoplasms: The 2008 World Health Organization criteria and point-of-care diagnostic algorithms

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