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.

The prognostic value of multiparametric cardiac magnetic resonance in patients with systemic light chain amyloidosis.

Background: Late gadolinium enhancement (LGE) is a classic imaging modality derived from cardiac magnetic resonance (CMR), which is commonly used to describe cardiac tissue characterization. T1 mapping with extracellular volume (ECV) and native T1 are novel quantitative parameters. The prognostic value of multiparametric CMR in patients with light chain (AL) amyloidosis remains to be thoroughly investigated.

Characteristics of Engraftment Syndrome following Autologous Stem Cell Transplantation in Light Chain Amyloidosis with Renal Involvement.

Engraftment syndrome (ES) is a clinical complication that occurs during the neutrophil recovery phase following hematopoietic stem cell transplantation. The clinical features of ES in light chain (AL) amyloidosis remains to be thoroughly investigated. This study was conducted to better understand the characteristics of ES following autologous stem cell transplantation (ASCT) in AL amyloidosis with renal involvement.

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.

Spectrum and prognosis of renal histopathological lesions in patients with inflammatory bowel disease: a cross-sectional study from a single center in China.

To explore the spectrum, clinicopathological features and prognosis of patients with inflammatory bowel disease (IBD) including Crohn's disease (CD) and ulcerative colitis (UC) with renal involvement. Included in this cross-sectional study were 36 IBD patients, in whom renal biopsy was performed to analyze the histological pattern and prognosis. Renal histopathology was examined by light microscopy and immunofluorescence using the standard procedures.

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.

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.