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MYD88 wild‐type Waldenstrom Macroglobulinaemia: differential diagnosis, risk of histological transformation, and overall survival
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Personen und Körperschaften: | , , , , , , , , , , , , , , , , , , |
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Titel: |
MYD88 wild‐type Waldenstrom Macroglobulinaemia: differential diagnosis, risk of histological transformation, and overall survival |
In: | British Journal of Haematology, 180, 2018, 3, S. 374-380 |
veröffentlicht: |
Wiley
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Umfang: | 374-380 |
ISSN: |
0007-1048 1365-2141 |
DOI: | 10.1111/bjh.15049 |
Zusammenfassung: | <jats:title>Summary</jats:title><jats:p><jats:italic><jats:styled-content style="fixed-case">MYD</jats:styled-content>88</jats:italic> mutations are present in 95% of Waldenstrom Macroglobulinaemia (<jats:styled-content style="fixed-case">WM</jats:styled-content>) patients, and support diagnostic discrimination from other IgM‐secreting B‐cell malignancies. Diagnostic discrimination can be difficult among suspected wild‐type <jats:italic><jats:styled-content style="fixed-case">MYD</jats:styled-content>88</jats:italic> (<jats:italic><jats:styled-content style="fixed-case">MYD</jats:styled-content>88</jats:italic><jats:styled-content style="fixed-case"><jats:sup>WT</jats:sup></jats:styled-content>) <jats:styled-content style="fixed-case">WM</jats:styled-content> cases. We systematically reviewed the clinical, pathological and laboratory studies for 64 suspected <jats:italic><jats:styled-content style="fixed-case">MYD</jats:styled-content>88</jats:italic><jats:styled-content style="fixed-case"><jats:sup>WT</jats:sup> WM</jats:styled-content> patients. World Health Organization and <jats:styled-content style="fixed-case">WM</jats:styled-content> consensus guidelines were used to establish clinicopathological diagnosis. Up to 30% of suspected <jats:italic><jats:styled-content style="fixed-case">MYD</jats:styled-content>88</jats:italic><jats:styled-content style="fixed-case"><jats:sup>WT</jats:sup> WM</jats:styled-content> cases had an alternative clinicopathological diagnosis, including IgM multiple myeloma. The estimated 10‐year survival was 73% (95% confidence interval [<jats:styled-content style="fixed-case">CI</jats:styled-content>] 52–86%) for <jats:italic><jats:styled-content style="fixed-case">MYD</jats:styled-content>88</jats:italic><jats:styled-content style="fixed-case"><jats:sup>WT</jats:sup> </jats:styled-content><jats:italic>versus</jats:italic> 90% (95% <jats:styled-content style="fixed-case">CI</jats:styled-content> 82–95%) for mutated <jats:italic>(<jats:styled-content style="fixed-case">MYD</jats:styled-content>88</jats:italic><jats:styled-content style="fixed-case"><jats:sup>MUT</jats:sup></jats:styled-content>) <jats:styled-content style="fixed-case">WM</jats:styled-content> patients (Log‐rank <jats:italic>P</jats:italic> < 0·001). Multivariate analysis only showed <jats:italic><jats:styled-content style="fixed-case">MYD</jats:styled-content>88</jats:italic> mutation status (<jats:italic>P</jats:italic> < 0·001) as a significant determinant for overall survival. Diffuse large B‐cell lymphoma (<jats:styled-content style="fixed-case">DLBCL</jats:styled-content>) was diagnosed in 7 (15·2%) and 2 (0·76%) of <jats:italic><jats:styled-content style="fixed-case">MYD</jats:styled-content>88</jats:italic><jats:styled-content style="fixed-case"><jats:sup>WT</jats:sup></jats:styled-content> and <jats:italic><jats:styled-content style="fixed-case">MYD</jats:styled-content>88</jats:italic><jats:styled-content style="fixed-case"><jats:sup>MUT</jats:sup></jats:styled-content> patients, respectively (Odds ratio 23·3; 95% <jats:styled-content style="fixed-case">CI</jats:styled-content> 4·2–233·8; <jats:italic>P</jats:italic> < 0·001). Overall survival was shorter among <jats:italic><jats:styled-content style="fixed-case">MYD</jats:styled-content>88</jats:italic><jats:styled-content style="fixed-case"><jats:sup>WT</jats:sup></jats:styled-content> patients with an associated <jats:styled-content style="fixed-case">DLBCL</jats:styled-content> event (Log‐rank <jats:italic>P</jats:italic> = 0·08). The findings show that among suspected <jats:italic><jats:styled-content style="fixed-case">MYD</jats:styled-content>88</jats:italic><jats:styled-content style="fixed-case"><jats:sup>WT</jats:sup> WM</jats:styled-content> cases, an alternative clinicopathological diagnosis is common and can impact clinical care. <jats:styled-content style="fixed-case">WM</jats:styled-content> patients with <jats:italic><jats:styled-content style="fixed-case">MYD</jats:styled-content>88</jats:italic><jats:styled-content style="fixed-case"><jats:sup>WT</jats:sup></jats:styled-content> disease have a high incidence of associated <jats:styled-content style="fixed-case">DLBCL</jats:styled-content> events and significantly shorter survival <jats:italic>versus</jats:italic> those with <jats:italic><jats:styled-content style="fixed-case">MYD</jats:styled-content>88</jats:italic><jats:styled-content style="fixed-case"><jats:sup>MUT</jats:sup></jats:styled-content> disease.</jats:p> |
Format: | E-Article |
Quelle: | Wiley (CrossRef) |
Sprache: | Englisch |