FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2344594786> ?p ?o ?g. }

• W2344594786 endingPage "2084" @default.
• W2344594786 startingPage "2082" @default.
• W2344594786 abstract "Primary plasma cell leukemia (PPCL) is one of the most challenging diseases for hematologists. Despite the younger age of patients with PPCL, we have not obtained, in this rare and aggressive disorder, the same important therapeutic progress seen during past years in multiple myeloma (MM). Nevertheless, a registry study of 445 patients with PPCL showed a modest, but statistically significant improvement in overall survival (OS), which has increased from 5 months in patients diagnosed between 1973 and 2005 to 12 months in those diagnosed between 2006 and 2009 after the introduction of the so-called novel agents (thalidomide, lenalidomide, and bortezomib) in the upfront setting. The most significant benefit was observed in older patients in whom the early mortality rate decreased from 26% to 15%. Literature about PPCL treatment is limited, sometimes conflicting, and often based on case reports or small retrospective series that frequently include both younger and elderly patients or secondary forms of plasma cell leukemia, which is a terminal event in previously diagnosed MM. Further confusion is caused by the reporting of heterogeneous drug combinations and transplantation procedures often collected over long periods that do not reflect contemporary practice. No randomized trials have been performed in PPCL thus far. Studies have suggested that regimens that contain bortezomib, the first-in-class proteasome inhibitor (PI), induce an overall response rate (ORR) of 69% to 89.9% in PPCL, with at least very good partial response (VGPR) in 28.5% to 37.9% of patients and a median OS that ranges from 12.6 months to about 2 years. These results generally compared favorably with PPCL series not treated with bortezomib-based regimens. Other groups, however, did not find a significant improvement in clinical outcome when novel drugs, particularly bortezomib, were used. Among immunomodulatory drugs, lenalidomide was more consistently investigated with low-dose dexamethasone in the first prospective trial in PPCL, which enrolled 23 patients with a new diagnosis (median age, 60 years). In this phase II study, ORR to induction treatment was 74% (VGPR or better, 39%). Four of five responders not eligible for transplantation who started maintenance with low-dose lenalidomide relapsed within 1 year. Nine patients underwent autologous stem cell transplantation (SCT) after induction. With a median follow-up of 34 months, median progression-free survival (PFS) was 14 months, and median OS was 28 months. However, PFS and OS were 27 months and not reached in patients who underwent autologous SCTupfront versus 2 months and 12 months in those who did not, respectively. Three registry studies in 711 patients who underwent transplantation between 1980 and 2009 explored the role of autologous SCT in PPCL and showed that this procedure induced higher rates of complete response than in MM. Notwithstanding, autologous SCT in PPCL was less effective than in MM in the long term due to increased nonrelapse-relatedmortality and short duration of post-transplantation response; particularly, PFS was 14.3 months (median), 34% to 38% at 3 years, and 10% at 5 years, whereas OS was 25.7 months (median), 39.5% to 64% at 3 years, and 25% to 27.2% at 5 years. Furthermore, there was a trend toward superior OS in patients who underwent a double versus single autologous SCT. Other groups reported median OS of 34 to 38 months in patients with PPCL who underwent autologous SCT. Variable efficacy and feasibility of allogeneic SCT have been described in small retrospective series. In addition, two registry studies compared 135 patients with PPCL who underwent allogeneic SCT, with similar populations treated with autologous SCT. Allogeneic SCT was associated with a lower relapse rate but a much higher risk of nonrelapse-related mortality compared with autologous SCT, without evidence of survival benefits. Reported PFS and OS in patients with allografts were 20% and 39% at 3 years and 20% and 32% at 4 years, respectively; OS at 5 years was 19% for reduced-intensity conditioning and 27% for myeloablative conditioning allogeneic SCT. Of note, patients treated with allogeneic SCT plateaued at approximately 20%, similar to that seen in MM but at a lower level. In the article that accompanies this editorial, Royer et al report the second prospective phase II clinical trial in newly diagnosed PPCL, where novel agents and transplantation procedures were combined. Forty patients (median age, 57 years) were planned to receive induction therapy with four alternate cycles of bortezomib, dexamethasone, pegylated doxorubicin or bortezomib, dexamethasone, cyclophosphamide. ORR was 69%; complete response, 10%; and VGPR, 26%. Sixteen responding patients younger than 66 years with an HLA-matched donor received consolidation with a tandem sequence of single autologous SCT followed by reduced-intensity conditioning allogeneic SCT. Six" @default.
• W2344594786 created "2016-06-24" @default.
• W2344594786 creator A5044869604 @default.
• W2344594786 date "2016-06-20" @default.
• W2344594786 modified "2023-09-23" @default.
• W2344594786 title "Progress in the Treatment of Primary Plasma Cell Leukemia" @default.
• W2344594786 cites W1529714767 @default.
• W2344594786 cites W1549044564 @default.
• W2344594786 cites W1818751554 @default.
• W2344594786 cites W1967025604 @default.
• W2344594786 cites W1978930370 @default.
• W2344594786 cites W1988113294 @default.
• W2344594786 cites W1995048348 @default.
• W2344594786 cites W2002324468 @default.
• W2344594786 cites W2003426156 @default.
• W2344594786 cites W2003599195 @default.
• W2344594786 cites W2058966655 @default.
• W2344594786 cites W2060957671 @default.
• W2344594786 cites W2070653024 @default.
• W2344594786 cites W2082169216 @default.
• W2344594786 cites W2086442724 @default.
• W2344594786 cites W2095561966 @default.
• W2344594786 cites W2096497783 @default.
• W2344594786 cites W2099349054 @default.
• W2344594786 cites W2101761743 @default.
• W2344594786 cites W2111566607 @default.
• W2344594786 cites W2115565576 @default.
• W2344594786 cites W2163761092 @default.
• W2344594786 cites W2171889148 @default.
• W2344594786 cites W2181080337 @default.
• W2344594786 cites W2261861493 @default.
• W2344594786 cites W2344590234 @default.
• W2344594786 cites W4255559492 @default.
• W2344594786 cites W2080281636 @default.
• W2344594786 doi "https://doi.org/10.1200/jco.2016.66.6115" @default.
• W2344594786 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/27114588" @default.
• W2344594786 hasPublicationYear "2016" @default.
• W2344594786 type Work @default.
• W2344594786 sameAs 2344594786 @default.
• W2344594786 citedByCount "16" @default.
• W2344594786 countsByYear W23445947862016 @default.
• W2344594786 countsByYear W23445947862017 @default.
• W2344594786 countsByYear W23445947862018 @default.
• W2344594786 countsByYear W23445947862019 @default.
• W2344594786 countsByYear W23445947862021 @default.
• W2344594786 countsByYear W23445947862022 @default.
• W2344594786 countsByYear W23445947862023 @default.
• W2344594786 crossrefType "journal-article" @default.
• W2344594786 hasAuthorship W2344594786A5044869604 @default.
• W2344594786 hasConcept C121332964 @default.
• W2344594786 hasConcept C126322002 @default.
• W2344594786 hasConcept C1276947 @default.
• W2344594786 hasConcept C143998085 @default.
• W2344594786 hasConcept C2776364478 @default.
• W2344594786 hasConcept C2777977315 @default.
• W2344594786 hasConcept C2778461978 @default.
• W2344594786 hasConcept C2780353925 @default.
• W2344594786 hasConcept C502942594 @default.
• W2344594786 hasConcept C71924100 @default.
• W2344594786 hasConceptScore W2344594786C121332964 @default.
• W2344594786 hasConceptScore W2344594786C126322002 @default.
• W2344594786 hasConceptScore W2344594786C1276947 @default.
• W2344594786 hasConceptScore W2344594786C143998085 @default.
• W2344594786 hasConceptScore W2344594786C2776364478 @default.
• W2344594786 hasConceptScore W2344594786C2777977315 @default.
• W2344594786 hasConceptScore W2344594786C2778461978 @default.
• W2344594786 hasConceptScore W2344594786C2780353925 @default.
• W2344594786 hasConceptScore W2344594786C502942594 @default.
• W2344594786 hasConceptScore W2344594786C71924100 @default.
• W2344594786 hasIssue "18" @default.
• W2344594786 hasLocation W23445947861 @default.
• W2344594786 hasLocation W23445947862 @default.
• W2344594786 hasOpenAccess W2344594786 @default.
• W2344594786 hasPrimaryLocation W23445947861 @default.
• W2344594786 hasRelatedWork W171303446 @default.
• W2344594786 hasRelatedWork W2029070213 @default.
• W2344594786 hasRelatedWork W2071815761 @default.
• W2344594786 hasRelatedWork W2099429225 @default.
• W2344594786 hasRelatedWork W2123763668 @default.
• W2344594786 hasRelatedWork W2135530131 @default.
• W2344594786 hasRelatedWork W2152029039 @default.
• W2344594786 hasRelatedWork W3134462279 @default.
• W2344594786 hasRelatedWork W3195155777 @default.
• W2344594786 hasRelatedWork W4253312912 @default.
• W2344594786 hasVolume "34" @default.
• W2344594786 isParatext "false" @default.
• W2344594786 isRetracted "false" @default.
• W2344594786 magId "2344594786" @default.
• W2344594786 workType "article" @default.