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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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- Sahlberg, Arne, et al.
(författare)
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Forward modeling of pile-up events in liquid scintillator detectors for neutron emission spectroscopy
- 2021
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Ingår i: Review of Scientific Instruments. - : American Institute of Physics (AIP). - 0034-6748 .- 1089-7623. ; 92:8
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Tidskriftsartikel (refereegranskat)abstract
- When using liquid scintillator detectors to measure the neutron emission spectrum from fusion plasmas, the problem of pile-up distortion can be significant. Because of the large neutron rates encountered in many fusion experiments, some pile-up distortion can remain even after applying traditional pile-up elimination methods, which alters the shape of the measured light-yield spectrum and influences the spectroscopic analysis. Particularly, pile-up events appear as a high-energy tail in the measured light-yield spectrum, which obfuscates the contribution that supra-thermal ions make to the energy spectrum. It is important to understand the behavior of such “fast ions” in fusion plasmas, and it is hence desirable to be able to measure their contribution to the neutron spectrum as accurately as possible. This paper presents a technique for incorporating distortion from undetected pile-up events into the analysis of the light-yield spectrum, hence compensating for pile-up distortion. The spectral contribution from undetected pile-up events is determined using Monte Carlo methods and is included in the spectroscopic study as a pile-up component. The method is applied to data from an NE213 scintillator detector at JET and validated by comparing with results from the time-of-flight spectrometer TOFOR, which is not susceptible to pile-up distortion. Based on the results, we conclude that the suggested analysis method helps counteract the problem of pile-up effects and improves the possibilities for extracting accurate fast-ion information from the light-yield spectrum.
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