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- Hernandez-Molina, Gabriela, et al.
(författare)
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Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies
- 2022
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Ingår i: Rheumatology. - : Oxford University Press. - 1462-0324 .- 1462-0332. ; 62:1, s. 243-255
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Tidskriftsartikel (refereegranskat)abstract
- ObjectiveTo characterize 414 patients with primary SS who developed haematological malignancies and to analyse how the main SS- and lymphoma-related features can modify the presentation patterns and outcomes.MethodsBy January 2021, the Big Data Sjögren Project Consortium database included 11 966 patients fulfilling the 2002/2016 classification criteria. Haematological malignancies diagnosed according to the World Health Organization (WHO) classification were retrospectively identified.ResultsThere were 414 patients (355 women, mean age 57 years) with haematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). A total of 376 (91%) patients had mature B-cell malignancy, nearly half had extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT lymphoma) (n = 197), followed by diffuse large B-cell lymphoma (DLBCL) (n = 67), nodal MZL lymphoma (n = 29), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) (n = 19) and follicular lymphoma (FL) (n = 17). Rates of complete response, relapses and death were 80%, 34% and 13%, respectively, with a 5-year survival rate of 86.5% after a mean follow-up of 8 years. There were significant differences in age at diagnosis (younger in MALT, older in CLL/SLL), predominant clinical presentation (glandular enlargement in MALT lymphoma, peripheral lymphadenopathy in nodal MZL and FL, constitutional symptoms in DLBCL, incidental diagnosis in CLL/SLL), therapeutic response (higher in MALT lymphoma, lower in DLBCL) and survival (better in MALT, nodal MZL and FL, worse in DLBCL).ConclusionIn the largest reported study of haematological malignancies complicating primary SS, we confirm the overwhelming predominance of B-cell lymphomas, especially MALT, with the salivary glands being the primary site of involvement. This highly-specific histopathological scenario is linked with the overall good prognosis with a 5-year survival rate of nearly 90%.
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| 2. |
- Salmi, Tuomo, et al.
(författare)
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Neutron star parameter constraints for accretion-powered millisecond pulsars from the simulated IXPE data
- 2021
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Ingår i: Astronomy and Astrophysics. - : EDP SCIENCES S A. - 0004-6361 .- 1432-0746. ; 646
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Tidskriftsartikel (refereegranskat)abstract
- We have simulated the X-ray polarization data that can be obtained with the Imaging X-ray Polarimetry Explorer, when observing accretion-powered millisecond pulsars. We estimated the necessary exposure times for SAX J1808.4-3658 in order to obtain different accuracy in the measured time-dependent Stokes profiles integrated over all energy channels. We found that the measured relative errors strongly depend on the relative configuration of the observer and the emitting hotspot. The improvement in the minimum relative error in Stokes Q and U parameters as a function of observing time t scales as 1= p t,, and it spans the range from 30-90% with a 200 ks exposure time to 20-60% with a 500 ks exposure time (in the case of data binned in 19 phase bins). The simulated data were also used to predict how accurate measurements of the geometrical parameters of the neutron star can be made when modelling only Q and U parameters, but not the flux. We found that the observer inclination and the hotspot co-latitude could be determined with better than 10 degrees accuracy for most of the cases we considered. In addition, we show that the position of a secondary hotspot can also be constrained when the spot is not obscured by an accretion disc. These measurements can be used to further constrain the neutron star mass and radius when combined with modelling of the X-ray pulse profile.
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