Serum free light chain level-based and non-fixed cycle daratumumab treatment strategy for patients with light chain amyloidosis.

Background: In recent years, daratumumab (DARA) has gained widespread use in the treatment of systemic light chain (AL) amyloidosis. In this study, we assessed the efficacy and safety of a DARA treatment strategy based on serum free light chain (sFLC) levels and non-fixed cycles.

Methods: The study included 123 patients with Al amyloidosis who received DARA at our center between July 2020 and September 2023.

Acute kidney injury during autologous stem cell transplantation in light chain amyloidosis with kidney involvement and their impact on prognosis.

Acute kidney injury (AKI) is a complication related to important organ dysfunction during autologous stem cell transplantation (ASCT) in light chain (AL) amyloidosis. This study aims to validate the risk factors of AKI during different periods of ASCT and the impact of AKI on long-term outcomes. 302 patients with AL amyloidosis and kidney involvement who underwent ASCT were included.

Risk factors, treatments and outcomes of patients with light chain amyloidosis who relapse after autologous stem cell transplantation.

Relapse after ASCT is an important factor affecting the long-term prognosis of patients with AL amyloidosis. However, the risk factors of relapse are unknown and there are limited studies on treatment outcomes of these patients. We retrospectively reviewed 170 patients with AL amyloidosis who underwent ASCT between 2010 and 2021.

Clinical features of anemia in membranous nephropathy patients: a Chinese cohort study.

Background: Anemia is a common complication in patients with progressive chronic kidney disease. This cohort study evaluated the prevalence, clinical features and prognosis of membranous nephropathy (MN) with anemia.

Methods: We retrospectively analyzed a cohort of MN patients diagnosed using renal biopsy between February 2012 and February 2018.

Long-term follow-up of autologous peripheral blood hematopoietic stem cell transplantation for refractory lupus nephritis-a series study of 20 patients.

Background: Autologous hematopoietic cell transplantation (ASCT) improves immunologic homeostasis in autoimmune diseases. ASCT-treated refractory lupus nephritis (LN) has been reported. Nevertheless, the long-term outcome of patients with refractory LN after ASCT remains unknown.

The clinical features and outcomes of systemic light chain amyloidosis with hepatic involvement.

Objectives: To evaluate the clinical characteristics and prognostic factors of hepatic systemic light chain (AL) amyloidosis.

Methods: Eighty-eight patients diagnosed AL amyloidosis with hepatic involvement between June 2004 and January 2019 were analysed retrospectively.

Results: The median age of the patients was 55 years old, and the male to female ratio was 2.

Clinicopathological features and outcomes of SLE patients with renal injury characterised by thrombotic microangiopathy.

Objectives: Non-immune complex (IC)-mediated renal thrombotic microangiopathy (TMA) has been reported in patients with systemic lupus erythematosus (SLE), but most studies included patients with both renal TMA and IC-mediated lupus nephritis (LN). In this study, the clinicopathological features and outcomes of renal injury characterised by only renal TMA were retrospectively analyzed.

Methods: Patients with glomerular and/or vascular TMA in the absence of subendothelial or epithelial immune deposits were screened from 2,332 biopsied of SLE patients.

The role of induction therapy before autologous stem cell transplantation in low disease burden AL amyloidosis patients.

Background: Induction therapy is recommended before autologous stem cell transplantation (ASCT) for AL amyloidosis patients with high disease burden [bone marrow plasma cells (BMPCs) > 10%], but the role of induction therapy before ASCT in patients with low disease burden (BMPCs ≤ 10%) is still unknown.

Methods: A total of 227 patients with AL amyloidosis were included in this study. Among 227 patients, 124 patients received bortezomib-based induction prior to ASCT and were defined as group A, 35 patients received other chemotherapeutic induction and were defined as group B, and the other 68 patients without induction were defined as group C.

Characterization of thromboelastography of patients with different pathological types of nephrotic syndrome.

To investigate the changes in blood coagulability as measured by thromboelastography (TEG) in patients with nephrotic syndrome of different etiologies as well as in patients with venous thromboembolic events (VTE).From January 2013 to October 2017, patients who were diagnosed as idiopathic membranous nephropathy (IMN), minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) were enrolled into this retrospective study in which their clinical characteristics, including TEG variables, were investigated. According to the presence or absence of VTE, the patients with IMN were divided into 2 groups of VTE and non-VTE.

A risk stratification for systemic immunoglobulin light-chain amyloidosis with renal involvement.

Renal involvement is found in about 70% of patients with systemic immunoglobulin light-chain (AL) amyloidosis. However, there is no risk stratification system specialized for renal AL concerning patients' survival. Galectin-3 (Gal-3) has been reported to portend poor prognosis in other renal diseases.

Autologous Hematopoietic Stem Cell Transplantation for Refractory Lupus Nephritis.

Background And Objectives: Our study evaluated the efficiency and safety of autologous hematopoietic stem cell transplantation treatment for patients with refractory lupus nephritis.

Design, Setting, Participants, & Measurements: From July 2011 to January 2015, a total of 22 patients with refractory lupus nephritis were enrolled in this study. Peripheral blood stem cells were mobilized with cyclophosphamide and granulocyte colony stimulating factor and reinfused after treatment with cyclophosphamide and antithymocyte globulin.

Complete remission of both immunoglobulin light chain amyloidosis and psoriasis after autologous hematopoietic stem cell transplantation: A case report.

Rationale: Immunoglobulin light chain amyloidosis (AL amyloidosis) is characterized by the deposition of abnormal amyloid protein produced by a pathological plasma cell clone in various organs and soft tissues. Hematopoietic stem cell transplantation (HSCT) is an effective way to treat AL amyloidosis. Psoriasis is a common autoimmune disease (AID) and HSCT is a potential treatment for severe AIDs.

Bortezomib with dexamethasone as first-line treatment for AL amyloidosis with renal involvement.

Although bortezomib has reported efficacy in light chain (AL) amyloidosis, the role of bortezomib in combination with dexamethasone (BD) as the first-line treatment for patients with AL amyloidosis has not been determined. We analyzed the outcomes of 72 consecutive unselected patients, which received primary therapy with BD in a single center. The patients were newly diagnosed with AL amyloidosis with renal (100%), cardiac (72%), hepatic (19%) or nervous system (10%) involvement and underwent a median of 2 (1-6) cycles of BD treatment.

The clinical features and outcomes of systemic AL amyloidosis: a cohort of 231 Chinese patients.

Background: Few data are available on the clinical features and outcomes of Chinese patients with systemic immunoglobulin light-chain (AL) amyloidosis. The aim of this study is to reveal the clinical picture and risk factors of disease progression in a large cohort of Chinese patients with AL amyloidosis.

Methods: Patients in the Jinling Hospital amyloidosis registry from 2003 to 2011 were studied.

Induction therapy with bortezomib and dexamethasone followed by autologous stem cell transplantation versus autologous stem cell transplantation alone in the treatment of renal AL amyloidosis: a randomized controlled trial.

Background: Although the use of bortezomib alone and in combination with steroids has shown efficacy in AL amyloidosis, its role in combination with high-dose melphalan and autologous stem cell transplantation (HDM/SCT) is unknown. In this study, we evaluated bortezomib in combination with dexamethasone (BD) for induction chemotherapy prior to HDM/SCT.

Methods: This was a single-center, prospective, randomized controlled trial comparing induction therapy consisting of two BD cycles followed by HDM/SCT (BD + HDM/SCT) with HDM/SCT alone in the treatment of patients with newly diagnosed AL amyloidosis.

p66Shc mediates high-glucose and angiotensin II-induced oxidative stress renal tubular injury via mitochondrial-dependent apoptotic pathway.

p66Shc, a promoter of apoptosis, modulates oxidative stress response and cellular survival, but its role in the progression of diabetic nephropathy is relatively unknown. In this study, mechanisms by which p66Shc modulates high-glucose (HG)- or angiotensin (ANG) II-induced mitochondrial dysfunction were investigated in renal proximal tubular cells (HK-2 cells). Expression of p66Shc and its phosphorylated form (p-p66Shc, serine residue 36) and apoptosis were notably increased in renal tubules of diabetic mice, suggesting an increased reactive oxygen species production.