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Background and purpose: Peripheral neuropathy is a frequent complication of brentuximab vedotin (BV), used in CD30+ lymphoma treatment. Classic BV‐induced neuropathy (BV‐CN) is a mild distal sensory axonal polyneuropathy. Severe BV‐induced inflammatory neuropathies (BV‐IN) have been described. BV‐IN contribute to lymphoma‐associated morbidity but might be immunotherapy‐responsive. Our primary objective was to evaluate the rate of BV‐IN. Our secondary objectives were to determine risk factors and warning signs. Methods: We conducted a retrospective cohort study on all patients treated with BV at our center between April 2014 and September 2021. Clinical, biological, and electrophysiological data were collected. BV‐induced neuropathy was defined as the occurrence of neuropathy up to 3 months after BV discontinuation. BV‐IN was defined with criteria adapted from European Academy of Neurology/Peripheral Nerve Society 2021 electrodiagnostic criteria for chronic inflammatory demyelinating polyradiculoneuropathy. Other neuropathies were classified as BV‐CN. Results: Among 83 patients, 41 (49%) developed neuropathy: 35 BV‐CN and 6 BV‐IN. Thus, the rate of BV‐IN was 7.2%. Compared to patients with BV‐CN, no predisposing factor was identified. However, patients with BV‐IN more frequently presented muscle weakness (67% vs. 5.7%, p < 0.05), gait disorders (83% vs. 20%, p < 0.05), or acute or subacute onset (67% vs. 14%, p < 0.05). BV‐IN was frequently more severe (Common Terminology Criteria for Adverse Events grade ≥3; 50% vs. 0%, p < 0.05). Four patients were treated with immunotherapy. Conclusions: Brentuximab vedotin‐induced neuropathy is an overlooked complication. Based on four easily identifiable "red flags", we provide an algorithm to help non‐neurologist physicians that care for BV‐treated patients to detect BV‐IN. The aim of the algorithm is to decrease the diagnostic and management delay of this disabling neuropathy. [ABSTRACT FROM AUTHOR]

Aims: Predicting mortality in severe AL cardiac amyloidosis is challenging due to elevated biomarker levels and limited thresholds for stratifying severe cardiac damage. Methods and results: This prospective, observational, cohort study included de novo, confirmed cardiac AL amyloidosis patients at the Henri Mondor National Reference Centre. The goal was to identify predictors of mortality to enhance prognostic stratification and improve informed decision‐making regarding therapy. Over the 12‐year study period, among the 233 patients included, 133 were NYHA III‐IV and 179 Mayo 2004 III. The independent predictors for mortality identified were hsTnT, NT‐proBNP, cardiac output, and conjugated bilirubin. A novel prognostic, conditional stratification, Mondor amyloidosis cardiac staging (MACS) was developed with biomarker cut‐off values for Stage 1: hsTnT ≤ 107 ng/L and NT‐proBNP ≤ 3867 ng/L (n = 77; 33%); for stage 2 NT‐proBNP > 3867 ng/L (n = 72; 30%). For stage 3, if troponin >107 ng/L, regardless of NT‐proBNP then CB 4 μmol/L, was added (n = 41; 17.5%) and stage 4: CB > 4 μmol/L (n = 43; 18.5%). The median overall survival was 8 months 95% CI [2–24]. At 1 year, 102 (44%) patients died and the Kaplan–Meier median survival with MACS Stage 1 was not reached, while stage 2 was 15.2 months (95% CI [11–18]) and stage 3, 6.6 months (95% CI [1–13]). Notably, among European stage II patients, 17.1%, n = 8 were MACS stage 3 and European stage IIIb 21.4% (n = 23) were MACS stage 4. Importantly, among European stage IIIb patients 42.2% (n = 29) were classified MACS stage 4 and 12.5% n = 9 were only MACS stage 2. Conclusions: The Mondor prognostic staging system, including conjugate bilirubin may significantly improve prognostic stratification for patients with severe cardiac amyloidosis. [ABSTRACT FROM AUTHOR]

Keywords: AL amyloidosis; cardiac amyloidosis; venetoclax; BH3 mimetics; apoptosis EN AL amyloidosis cardiac amyloidosis venetoclax BH3 mimetics apoptosis 674 677 4 05/03/21 20210501 NES 210501 Systemic light-chain (AL) amyloidosis has a poor prognosis, especially when progressive heart failure occurs. Venetoclax induces profound and sustained responses in patients with relapsed/refractory light-chain amyloidosis We report here the results of a single-centre retrospective study on consecutive R/R AL amyloidosis patients treated off-label with venetoclax in a French academic referral centre between February 2017 and January 2020. Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis, Tours, France, 18-22 April 2004. [Extracted from the article]

Eighteen patients received a dose of 20 mg at each daratumumab infusions, three patients received 10 mg and four patients did not receive dexamethasone because of dexamethasone induced cardiac failure. Systemic light-chain (AL) amyloidosis is a plasma cell disorder characterized by extracellular deposition of monoclonal immunoglobulin light chains leading to organ dysfunction. We assessed the efficacy of daratumumab in such AL patients with severe cardiac involvement by performing a single-center retrospective study that included consecutive patients with Mayo Clinic stage III AL treated with daratumumab from May 2017 to August 2019. However, patients with severe cardiac amyloidosis involvement, defined as stage IIIb by the Mayo classification with European modification, have been excluded from these studies and little data on daratumumab use are available for patients with severe cardiac involvement. [Extracted from the article]