Publications by authors named "Stephanie Harel"

Inflammation in Waldenström Macroglobulinemia (iWM) predicts outcomes after immuno-chemotherapy and BTK inhibitors, but its origin is unknown. Here, we unravel increased clonal hematopoiesis in iWM patients (61% versus 23% in non-inflammatory WM), suggesting a contribution of environmental cells to iWM.

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  • This study evaluated the effectiveness of cardiac magnetic resonance (CMR) imaging compared to other diagnostic markers in predicting mortality in patients with AL cardiac amyloidosis (CA).
  • Conducted on 176 patients with a median age of 68 years, the study found that high levels of NT-proBNP and troponin, along with low heart function measurements, were linked to increased mortality risk.
  • Extracellular volume (ECV) from CMR emerged as a strong independent predictor of all-cause mortality and heart failure hospitalizations, suggesting it can enhance prognostic assessments beyond the established Mayo Clinic staging.
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A second autologous stem-cell transplantation (ASCT2) is considered for relapsed multiple myeloma (RMM) patients showing prolonged response after a first ASCT. However, given breakthrough treatments like anti-CD38 and immunotherapy, its role remains debated. We conducted a real-life study in 10 French centers (1996-2017) involving 267 RMM patients receiving ASCT2.

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Background: Acute kidney injury (AKI) has been reported after CAR-T cells, but available data are limited. We sought to describe the incidence of AKI in a cohort of patients hospitalized in the intensive care unit (ICU) following CAR-T cell reinjection, identify the primary factors linked to the onset of AKI, and ascertain the key determinants associated with kidney outcomes and mortality.

Methods: We retrospectively analyzed 119 patients hospitalized in ICU after CAR-T cell therapy between 2017 and 2023.

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Background: Carfilzomib, a new proteasome inhibitor indicated for patients with relapsed/refractory myeloma, has been associated with cases of thrombotic microangiopathy (CFZ-TMA). The role of variants in the complement alternative pathway and therapeutic potential of complement blockade with eculizumab remain to be determined.

Methods: We report 37 cases of CFZ-TMA recorded in the French reference center for TMA with their clinical characteristics, genetic analysis and outcome according to treatments.

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  • Bispecific antibodies (BsAbs) are used to treat relapsed or refractory multiple myeloma, but frequent infections were noted during clinical trials, indicating a need for further real-world data on infection risks and outcomes.
  • A multicenter retrospective study involving 229 patients in France from December 2020 to February 2023 found a high incidence of infections, with 234 recorded cases, primarily affecting the respiratory system, and a hospitalization rate of 56%.
  • The study identified corticosteroids used for managing cytokine release syndrome as a risk factor for infections, while using GPRC5D-targeting BsAbs and antibacterial prophylaxis was associated with a reduced risk.
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Background: Both light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis are types of cardiac amyloidosis (CA) that require accurate prognostic stratification to plan therapeutic strategies and follow-ups. Cardiac biomarkers, e.g.

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  • Renal impairment is frequent in multiple myeloma and negatively impacts survival; the main cause of kidney injury in these patients is cast nephropathy, which happens due to the precipitation of monoclonal free light chains in renal tubules.
  • Early detection and treatment of acute kidney injury are crucial to prevent it from becoming chronic, involving methods like rehydration and adjusting treatments for nephrotoxicity.
  • The standard treatment involves a combination of Bortezomib and Dexamethasone, but monitoring is essential, especially in patients with existing kidney issues, requiring collaboration between hematologists and nephrologists.
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Monoclonal immunoglobulin light chain (LC) crystalline inclusions within podocytes are rare, poorly characterized entities. To provide more insight, we now present the first clinicopathologic series of LC crystalline podocytopathy (LCCP) encompassing 25 patients (68% male, median age 56 years). Most (80%) patients presented with proteinuria and chronic kidney disease, with nephrotic syndrome in 28%.

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Chimeric antigen receptor T cells (CAR-T) have provided promising results in multiple myeloma (MM). However, many patients still relapse, pointing toward the need of improving this therapy. Here, we analyzed peripheral blood T cells from MM patients at different stages of the disease and investigated their phenotype and capacity to generate functional CAR-T directed against CS1 or B Cell Maturation antigen.

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  • CAR-T cell therapy is a promising treatment for difficult-to-treat blood cancers, but critical care management is vital for patients undergoing this therapy.
  • A study analyzed 71 patients who required ICU admission after receiving CAR-T cell therapy, revealing that most had experiences such as cytokine release syndrome and infections, with shock being the main reason for admission.
  • The study found that significant survival rates were possible, with patient outcomes heavily influenced by the reasons for ICU admission and the level of organ dysfunction.
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Waldenström's macroglobulinaemia (WM) is a B-cell neoplasm resulting from bone marrow lymphoplasmacytic infiltration and monoclonal IgM secretion. Some patients present concomitant inflammatory syndrome attributed to the disease activity; we named this syndrome inflammatory WM (IWM). We retrospectively analysed all WM patients seen in a single tertiary referral centre from January 2007 to May 2021, and after excluding aetiologies for the inflammatory syndrome using a pertinent blood workup, including C-reactive protein (CRP), and imaging, we identified 67 (28%) IWM, 166 (68%) non-IWM, and nine (4%) WM with inflammatory syndrome of unknown origin.

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  • There hasn't been much focus on how to manage the risks of blood clots and bleeding in people with a condition called systemic amyloidosis.
  • Studies show that around 5-10% of patients with amyloidosis have issues with blood clots, especially if their hearts are affected, which can make their health worse.
  • Common problems that increase the risk of clots include heart failure and a heart condition called atrial fibrillation, while the risk of bleeding can rise due to digestive problems and kidney issues.
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Multiple myeloma (MM) is rare in young patients, especially before age 40 years at diagnosis, representing <2% of all patients with MM. Little is known about the disease characteristics and prognosis of these patients. In this study, we examined 214 patients diagnosed with MM at age ≤40 years over 15 years, in the era of modern treatments.

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  • - The study aimed to identify specific patient profiles among COVID-19 patients with respiratory symptoms in order to assess their outcomes based on various characteristics at admission.
  • - Researchers conducted a retrospective analysis of 220 patients and identified three distinct clusters with differing health backgrounds and survival rates: Cluster 1 (older patients with cancers), Cluster 2 (older, overweight patients with hypertension and renal issues), and Cluster 3 (younger patients with gastrointestinal symptoms).
  • - The findings suggest that using a cluster approach can effectively help doctors predict patient prognosis and tailor treatment strategies based on these profiles.
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