Protein disulfide isomerase is essential for osteoblast differentiation in mice.

Protein disulfide isomerase (PDI) is an oxidoreductase responsible for the formation, reduction and isomerization of disulfide bonds of nascent proteins in endoplasmic reticulum (ER). So far, the role of PDI in bone biology has never been characterized using genetically-modified animal models. In this study we generated osteoblast- specific PDI-deficient mice by crossing PDI-floxed (PDI) mice with Osx-Cre mice.

Exploration of risk factors and characteristics of COVID-19 infection among patients with hematological malignancies in Suzhou, China: a retrospective study.

Background And Aim: Patients diagnosed with cancer, particularly those with hematologic malignancies, frequently exhibit a state of immunosuppression. Currently, there remains a scarcity of dependable biomarkers for assessing the severity of COVID-19 in individuals with hematologic malignancies. We conducted a retrospective study of morbidity and mortality in patients with hematological malignancies (HM) who had contracted COVID-19.

[Efficacy and Safety of Venetoclax in Combination with Hypomethylating Agents for the Treatment of High-Risk Myelodysplastic Syndromes].

Objective: To investigate the clinical efficacy and safety of venetoclax (VEN) in combination with hypomethylating agent (HMA) in the treatment of patients with high-risk myelodysplastic syndromes (MDS).

Methods: A total of 30 patients with high-risk MDS who received the combination of VEN and HMA from March 2019 to November 2022 were included. The overall response rate (ORR), modified overall response rate (mORR), overall survival (OS), progression-free survival (PFS), and adverse events of all included patients were evaluated.

Venetoclax and Decitabine vs Intensive Chemotherapy as Induction for Young Patients with Newly Diagnosed AML.

Venetoclax combined with hypomethylating agents is approved for frontline therapy in older/unfit acute myeloid leukemia (AML) patients. However, prospective data on this low intensity therapy in treatment-naive younger AML patients are lacking. This study investigated the efficacy and safety of venetoclax plus decitabine (VEN-DEC) as induction in untreated young fit AML patients in a randomized trial.

Cladribine Added to Idarubicin and Cytarabine as an Induction Regimen for Patients with De Novo Acute Myeloid Leukemia: A Multicenter, Randomized Phase III Trial.

Purpose: To assess the efficacy and safety of an induction regimen composed of idarubicin, cytarabine, and cladribine (IAC) in patients with de novo acute myeloid leukemia (AML).

Patients And Methods: Adult patients with newly diagnosed AML were randomized to the IAC group (cladribine 5 mg/m2/day for 5 days, idarubicin 8 mg/m2/day for 3 days, and cytarabine 100 mg/m2/day for 7 days) and the IA group (idarubicin 12 mg/m2/day for 3 days and cytarabine 100 mg/m2/day for 7 days) at a 1:2 ratio. The primary endpoint was complete remission (CR) after induction.

Venetoclax in combination with hypomethylating agents in newly diagnosed and relapsed β acute myeloid leukemia.

Not available.

A 2D-STI echocardiographic diagnostic model established for cardiac amyloidosis complicated with multiple myeloma.

Objective: This study aimed to investigate the clinical characteristics of multiple myeloma(MM) with amyloidosis(AL) and evaluate the diagnostic utility of two-dimensional speckle tracking imaging(2D-STI) echocardiography in MM with cardiac amyloidosis(CA), with the goal of providing guidance for early screening and differentiation.

Methods: Among 616 newly diagnosed multiple myeloma (NDMM) patients, 359 met the inclusion and exclusion criteria, and divided into MM-AL and MM without AL according to the histopathological results. MM-AL patients were subdivided into MM-CA and MM without CA subgroups according to cardiac involvement criteria.

A comparison of porcine anti-human T lymphocyte immunoglobulin, rabbit-ATG for GVHD prophylaxis and without ATG in matched sibling donor transplantation.

Introduction: Research on anti-lymphocyte globulins other than rabbit anti-thymocyte globulin (r-ATG) in prevention of graft-versus-host-disease (GVHD) following HLA-matched siblings hematopoietic stem cell transplantation (MSD-HSCT) is limited. The objective of this study is to investigate the distinct impacts of porcine anti-human T lymphocyte immunoglobulin (p-ATG) and r-ATG on outcomes of MSD-HSCT in patients with hematologic malignancies.

Patients And Methods: This retrospective analysis enrolled 373 consecutive patients who underwent MSD-HSCT from January 2019 to October 2023.

Differential transport pathways of saturated and unsaturated fatty acid esters in male mouse hepatocytes.

Saturated fatty acid (SFA) and unsaturated fatty acid (UFA) have distinct impacts on health. Whether SFA and UFA are differentially transported in liver remains elusive. Here, we find the secretion of UFA but not SFA esters is retarded in a male mouse hepatic endoplasmic reticulum (ER) stress model.

Usp11 maintained the survival of marginal zone B cells under ionizing radiation by deubiquitinating DLL1 and JAG2.

Efficacy of radiation therapy is compromised by hematopoietic and immune impairments, with elusive underlying causes. This study aimed to elucidate Usp11's role in radiation-induced injuries and uncover related mechanisms. Utilized ARS mouse model to observe survival rates of Usp11 (KO) mice post-TBI (Total Body Irradiation).

Extracellular thiol isomerase ERp5 regulates integrin αIIbβ3 activation by inhibition of fibrinogen binding.

Recent studies have shown that anti-ERp5 antibodies inhibit platelet activation and thrombus formation; Moreover, ERp5-deficient platelets exhibit enhanced platelet reactivity via regulation of endoplasmic reticulum (ER) stress. In this study, we used a new ERp5-knockout mouse model as well as recombinant ERp5 (rERp5) protein, to examine the role of ERp5 in platelet function and thrombosis. Although platelet-specific ERp5-deficient mice had decreased platelet count, the mice had shortened tail-bleeding times and enhanced platelet accumulation in FeCl-induced mesenteric artery injury, compared with wild-type mice.

CPSF6-RARγ interacts with histone deacetylase 3 to promote myeloid transformation in RARG-fusion acute myeloid leukemia.

Acute myeloid leukemia (AML) with retinoic acid receptor gamma (RARG) fusions, which exhibits clinical features resembling acute promyelocytic leukemia (APL), has been identified as a new subtype with poor clinical outcomes. The underlying mechanism of RARG-fusion leukemia remains poorly understood, and needs to be explored urgently to instruct developing effective therapeutic strategies. Here, using the most prevalent RARG fusion, CPSF6-RARG (CR), as a representative, we reveal that the CR fusion, enhances the expansion of myeloid progenitors, impairs their maturation and synergizes with RAS mutations to drive more aggressive myeloid malignancies.

[Clinical Characteristics of Pneumocystis Jiroveci Pneumonia after Allogeneic Hematopoietic Stem Cell Transplantation].

Objective: To summarize the clinical characteristics of patients with combined pneumocystis jiroveci pneumonia (PJP) after allogeneic hematopoietic stem cell transplantation (allo-HSCT).

Methods: The clinical manifestations, laboratory tests, imaging findings, and treatment outcomes of 21 allo-HSCT patients with PJP diagnosed at the First Affiliated Hospital of Soochow University and Soochow Hopes Hematology Hospital from July 2018 to July 2023 were retrospective analyzed.

Results: Among the 21 patients, the male -to-female ratio was 2.

Impaired megakaryopoiesis due to aberrant macrophage polarization via BTK/Rap1/NF-κB pathway in sepsis-induced thrombocytopenia.

Sepsis-induced thrombocytopenia (SIT) is a widely accepted predictor of poor prognosis during sepsis, while the mechanism of SIT remains elusive. In this study, we revealed that SIT patients and septic mice exhibited higher levels of pro-inflammatory macrophages and phosphorylated Bruton's tyrosine kinase (p-BTK) expression in macrophages, which were closely correlated with platelet counts. Treatment with the BTK inhibitor BGB-3111 in SIT mice resulted in enhanced production of megakaryocytes and platelets.

Case report: CAR-T therapy demonstrated safety and efficacy in relapsed/refractory diffuse large B-cell lymphoma patients complicated with hepatitis B-related cirrhosis.

Chimeric antigen receptor T-cell (CAR-T) therapy has demonstrated both efficacy and safety in relapsed/refractory diffuse large B-cell lymphoma (DLBCL) patients infected with hepatitis B virus (HBV). However, its applicability in individuals with liver cirrhosis remains largely unexplored due to the potential for unpredictable complications. Here, we report three cases (P1, P2, and P3) of relapsed/refractory DLBCL with HBV-related cirrhosis treated with CAR-T cell infusion.

Clinical outcomes and safety of CAR-T cells in treatment of T-Cell acute lymphoblastic leukemia/lymphoma.

Relapsed or refractory T-cell acute lymphoblastic leukemia/lymphoma (r/r T-ALL/LBL) are frequently aggressive and associated with unfavorable prognoses. Pan-targeted Chimeric Antigen Receptor (CAR) T-cell therapy have shown promising results in clinical trials. In recent years, CD7 CAR T-cell and CD5 CAR T-cell demonstrate effectiveness in treating r/r T-ALL/LBL patients with bone marrow infiltration.

Efficacy of emapalumab in the management of anti‑CD19 chimeric antigen receptor T‑cell therapy‑associated cytokine release syndrome: A report of two cases.

Chimeric antigen receptor (CAR) T-cell therapy is an effective treatment for diffuse large B-cell lymphoma (DLBCL). However, it may activate the systemic immune system of the patient, resulting in cytokine release syndrome (CRS). Emapalumab is a human monoclonal antibody targeting interferon-γ, inhibiting its interaction with cell surface receptors and the subsequent activation of inflammatory pathways.

Dimethyl fumarate ameliorates chronic graft-versus-host disease by inhibiting Tfh differentiation via Nrf2.

Chronic graft-versus-host disease (cGVHD), characterized by chronic tissue inflammation and fibrosis involving multiple organs, remains a major complication after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Dimethyl fumarate (DMF) is an anti-inflammatory drug approved for the treatment of multiple sclerosis and psoriasis. We previously reported that DMF effectively inhibits acute GVHD (aGVHD) while preserving the graft-versus-leukemia effect.