Efficacy of anifrolumab on systemic lupus erythematosus patients with serological manifestations: A post hoc analysis of the Japan subgroup of the TULIP-2 Trial.

Objectives: To describe the efficacy of anifrolumab vs. placebo in Japanese systemic lupus erythematosus (SLE) patients with low complement (C3 or C4) and/or who are positive for anti-double stranded DNA (anti-dsDNA) antibodies.

Methods: This was a descriptive post hoc analysis of Japanese SLE patients with serological manifestations in the TULIP-2 trial who received either anifrolumab or placebo.

Cluster analysis identifies the differential impact of disease activity and severity on functional status and patient satisfaction in rheumatoid arthritis: the FRANK registry.

Objectives: The purpose of the present study was to investigate the differential impact of disease activity and severity on functional status and patient satisfaction in rheumatoid arthritis (RA) using cluster analysis on data from the FRANK registry.

Methods: Data from 3,619 RA patients in the FRANK registry were analysed. Patients were grouped using hierarchical and k-means cluster analyses based on age, physician's global assessment (PhGA), patient's pain assessment (PtPA), and Steinbrocker stage.

Disease activity and glucocorticoid tapering patterns in Japanese patients with systemic lupus erythematosus treated with anifrolumab: post hoc analysis of the Japanese subpopulation of the TULIP-2 study.

Objectives: To investigate the efficacy of anifrolumab on disease activity and glucocorticoid tapering patterns in Japanese patients with systemic lupus erythematosus (SLE).

Methods: We analysed disease activity and glucocorticoid tapering in the Japanese subpopulation (anifrolumab, n = 24; placebo, n = 19) of the TULIP-2 trial, which showed the efficacy and safety of anifrolumab in patients with moderate-to-severe active SLE.

Results: The percentage of patients who achieved a British Isles Lupus Assessment Group-based Composite Lupus Assessment response at Week 52 was greater in the anifrolumab group than placebo [50.

Real-world Outcomes of Commercial Tisagenlecleucel for Children, Adolescents, and Young Adults With Acute Lymphoblastic Leukemia in Japan.

Chimeric antigen receptor (CAR) T cells are a major new treatment option for children, adolescents, and young adults (CAYA) patients with relapsed and refractory (R/R) B cell acute lymphoblastic leukemia (B-ALL). Therefore, accumulating evidence from real-world experiences of CAR-T outcomes in various regions worldwide is important, particularly when comparing outcomes of patients with differing medical and ethnic backgrounds. More than 5 years have passed since tisagenlecleucel was approved in Japan.

Femoral bone mineral density as a tool of personalized medicine for rheumatoid arthritis: Interleukin-6 inhibitors for patients with low density whereas tumor necrosis factor inhibitor for patients with preserved density?

Objectives: There is a lack of indicators to distinguish between interleukin-6 inhibitors responders and tumor necrosis factor inhibitors responders in the treatment of rheumatoid arthritis. Osteoporosis is a complication of rheumatoid arthritis and is closely related to inflammatory pathology. The purpose of this study was to evaluate whether bone mineral density can distinguish interleukin-6 inhibitors responders from tumor necrosis factor inhibitors responders in rheumatoid arthritis.

Azacitidine treatment for myeloid leukemia associated with Down syndrome: A nationwide retrospective study in Japan.

Hypomethylating agent treatment for myeloid leukemia associated with Down syndrome (ML-DS) has been scarcely reported. Herein, we collected information on azacitidine treatment for ML-DS in Japan. Forty-eight cycles of azacitidine treatment were performed for 12 patients, including 11 relapsed or refractory (R/R) patients.

Allogeneic Hematopoietic cell Transplantation Using Alemtuzumab in Asian Patients with Inborn Errors of Immunity.

Alemtuzumab is used with reduced-toxicity conditioning (RTC) in allogeneic hematopoietic cell transplantation (HCT), demonstrating efficacy and feasibility for patients with inborn errors of immunity (IEI) in Western countries; however, the clinical experience in Asian patients with IEI is limited. We retrospectively analyzed patients with IEI who underwent the first allogeneic HCT with alemtuzumab combined with RTC regimens in Japan. A total of 19 patients were included and followed up for a median of 18 months.

Analysis of overweight/obese pediatric patients with acute myeloid leukemia: a report from the Japanese Pediatric Leukemia/Lymphoma Study Group AML-05 study.

The dosage of chemotherapy drugs for overweight/obese children with acute myeloid leukemia (AML) has been empirically reduced based on ideal body weight (BW) in Japan to reduce the risk of adverse events. We investigated the associations between pre-therapeutic body mass index (BMI) and clinical outcomes among children with AML. A total of 280 children were divided into two groups based on the World Health Organization Child Growth Standards: a healthy-weight group (n = 254), and an overweight/obese group (n = 26).

[Sequential therapy with inotuzumab ozogamicin followed by CAR T-cell therapy for Philadelphia chromosome-negative acute lymphoblastic leukemia].

A 25-year-old woman with a history of B-cell acute lymphoblastic leukemia over ten years ago was referred to our hospital with a chief complaint of leukoblastosis. She was participating in a JPLSG (Japanese Pediatric Leukemia/Lymphoma Study Group) clinical study at that time. We diagnosed ALL relapse by multi-color flow cytometric analysis of bone marrow samples at admission, with reference to previous JPLSG data.

Impact of combined pulmonary fibrosis and emphysema on lung cancer risk and mortality in rheumatoid arthritis: A multicenter retrospective cohort study.

Objective: Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome characterized by the coexistence of emphysema and fibrotic interstitial lung disease (ILD). The aim of this study was to examine the effect of CPFE on lung cancer risk and lung cancer-related mortality in patients with rheumatoid arthritis (RA).

Methods: We conducted a multicenter retrospective cohort study of patients newly diagnosed with lung cancer at five community hospitals between June 2006 and December 2021.

Efficacy and safety of lascufloxacin for nursing- and healthcare-associated pneumonia: A single-arm, open-label clinical trial.

Background: Nursing- and healthcare-associated pneumonia (NHCAP) constitutes most of the pneumonia in elderly patients including aspiration pneumonia in Japan. Lascufloxacin (LSFX) possesses broad antibacterial activity against respiratory pathogens, such as Streptococcus spp. And anaerobes inside the oral cavity.

Eculizumab treatment in paediatric patients diagnosed with aHUS after haematopoietic stem cell transplantation: a HSCT-TMA case series from Japanese aHUS post-marketing surveillance.

Haematopoietic stem-cell transplantation (HSCT)-associated thrombotic microangiopathy (HSCT-TMA) is a serious complication with high mortality. Accumulating evidence suggests that complement dysregulation is potentially involved in the development of HSCT-TMA. We retrospectively analysed the clinical characteristics and outcomes of thirteen paediatric patients who were diagnosed with atypical haemolytic uremic syndrome and treated with eculizumab to manage HSCT-TMA during post-marketing surveillance in Japan.

Successful Treatment of a Patient with Drug-Refractory Rheumatoid Arthritis-Associated Interstitial Lung Disease with Upadacitinib: A Case Report.

Insufficient evidence exists regarding the efficacy of Janus kinase inhibitors (JAKis), a class of targeted synthetic disease-modifying anti-rheumatic drugs (tsDMARDs), in the treatment of rheumatoid arthritis (RA)-associated interstitial lung disease (ILD). Herein, we present a case of RA-ILD refractory to previous treatments that exhibited favorable response to upadacitinib. A 69-year-old man, former smoker, was diagnosed with RA-ILD based on persistent symmetric polyarthritis, elevated C-reactive protein levels and erythrocyte sedimentation rate, reduced diffusing capacity for carbon monoxide/alveolar volume (D 69.

Biologic and clinical features of childhood gamma delta T-ALL: identification of STAG2/LMO2 γδ T-ALL as an extremely high risk leukemia in the very young.

Purpose: Gamma delta T-cell receptor-positive acute lymphoblastic leukemia (γδ T-ALL) is a high-risk but poorly characterized disease.

Methods: We studied clinical features of 200 pediatric γδ T-ALL, and compared the prognosis of 93 cases to 1,067 protocol-matched non-γδ T-ALL. Genomic features were defined by transcriptome and genome sequencing.