A case of autoimmune factor XIII deficiency due to clearance-accelerating and inhibitory anti-FXIII autoantibodies.

A 63-year-old man, previously diagnosed with multiple autoimmune diseases, developed life-threatening bleeding after gastrectomy for stomach cancer. He survived due to treatment with factor XIII (FXIII) concentrates immediately after his FXIII antigen (Ag) level was reported to be < 5% of normal. Detailed examination by the Japanese Collaborative Research Group on autoimmune coagulation factor deficiencies revealed the presence of anti-FXIII-A and anti-FXIII-B subunit autoantibodies on immunoblot analyses, and thus autoimmune FXIII deficiency (AiF13D) was diagnosed based on the Japanese and international diagnostic criteria.

Pathogenic TNFRSF13B Variant in an Adult Japanese Patient with Common Variable Immunodeficiency.

Common variable immunodeficiency (CVID) is a primary B cell immunodeficiency disorder. Symptoms do not develop immediately after birth, and patients are often diagnosed in childhood and adulthood. These patients often develop autoimmune diseases and malignant tumors.

Red blood cell distribution width is a useful biomarker to predict bleeding and thrombosis risks in patients with immune thrombocytopenic purpura.

Bleeding and thrombosis are common complications during immune thrombocytopenic purpura (ITP) treatment. There is a strong need to predict bleeding and thrombosis risks before ITP treatment to optimize therapy and appropriately manage these complications. We performed a retrospective cohort study of 120 patients with primary ITP to identify a biomarker to predict bleeding and thrombosis.

Bleeding sites and treatment strategies for cardiac tamponade by catheter ablation requiring thoracotomy: risks of catheter ablation in patients with left atrial diverticulum.

Background: There is insufficient information regarding the bleeding sites and surgical strategies of cardiac tamponade during catheter ablation for atrial fibrillation (AF).

Case Presentation: Of the five patients with cardiac tamponade, three required surgical intervention and two required pericardiocentesis. In the first case of three cardiac tamponades requiring surgical intervention, considering that the peripheral route was used, the catecholamines did not reach the heart, and due to unstable vital signs, venoarterial extracorporeal membrane oxygenation (VA-ECMO) was inserted.

COVID-19 antibody titers after tixagevimab-cilgavimab injection in patients with hematologic diseases; a single-center, prospective study.

Knowledge of the SARS-CoV-2 antibody titers induced by tixagevimab-cilgavimab in patients with hematologic diseases remains insufficient. Here, we performed a single-center, prospective study to reveal the changes in antibody titer after administration of tixagevimab-cilgavimab in 78 patients with hematologic diseases. The median peak titer was 155.

Recurrence of solitary plasmacytoma in the liver 10 years after the onset of multiple bone lesions.

A 79-year-old man presented with a history of solitary plasmacytoma in the bone 10 years ago. Chemoradiotherapy was effective, and remission was maintained with intermittent treatment at relapse of the bone lesions. One year after the last treatment, a follow-up computed tomography (CT) scan revealed multiple liver masses, and a liver biopsy revealed plasmacytoma.

Sustained remission after cord blood transplantation for breast cancer with lung metastases and myelodysplastic syndrome.

Allogenic hematopoietic stem cell transplantation (allo-HSCT) is not a standard therapy for solid cancer because of its high toxicity and insufficient evidence levels. However, the potential graft-versus-solid-tumor (GVT) effect of this therapy has been discussed. Many case reports have also described treatment effects of allo-HSCT in patients with hematologic malignancies and active solid tumors.

Successful treatment of isolated central nervous system recurrence of primary testicular lymphoma by autologous stem cell transplantation using a conditioning regimen of thiotepa and busulfan.

Primary testicular lymphoma (PTL) frequently relapses in the central nervous system (CNS) despite prophylactic intrathecal chemotherapy, and the outcome for CNS recurrence of PTL is very poor. We report a case of isolated CNS recurrence of bilateral PTL. Our patient achieved complete response (CR) after rituximab-combination chemotherapy for PTL.

[Spontaneous regression of HIV-associated EBV-positive mucocutaneous ulcer due to immune reconstruction with antiretroviral therapy].

A 24-year-old man was found to have an ileocecal ulcer by colonoscopy. A pathological diagnosis of diffuse large B-cell lymphoma (DLBCL) with diffuse positive reaction of Epstein-Barr encoding region (EBER) by in situ hybridization was made based on analysis of the specimen. Acquired immunodeficiency syndrome (AIDS) complicated by pneumocystis jirovecii pneumonia was also diagnosed.

Clinical impact and characteristics of erythroid dysplasia in adult aplastic anaemia: Results from a multicentre registry.

Morphological dysplasia in haematopoietic cells, defined by a 10% threshold in each lineage, is one of the diagnostic criteria for myelodysplastic neoplasms. Dysplasia limited to the erythroid lineage has also been reported in some cases of aplastic anaemia (AA); however, its significance remains unclear. We herein examined the impact of erythroid dysplasia on immunosuppressive therapy responses and survival in AA patients.

Antifungal profile against Candida auris clinical isolates of tyroscherin and its new analog produced by the deep-sea-derived fungal strain Scedosporium apiospermum FKJ-0499.

A new antifungal compound, named N-demethyltyroscherin (1), was discovered from the static fungal cultured material of Scedosporium apiospermum FKJ-0499 isolated from a deep-sea sediment sample together with a known compound, tyroscherin (2). The structure of 1 was elucidated as a new analog of 2 by MS and NMR analyses. The absolute configuration of 1 was determined by chemical derivatization.

Granzyme B Expression in the Tumor Microenvironment as a Prognostic Biomarker for Patients with Triple-Negative Breast Cancer.

Tumor-infiltrating lymphocytes in the tumor microenvironment are important in the treatment of triple-negative breast cancer (TNBC). Cytotoxic T cells produce cytokines and cytotoxic factors, such as perforin and granzyme, which induce apoptosis by damaging target cells. To identify biomarkers of these cells, we investigated granzyme B (GZMB) in the tumor microenvironment as a biomarker of treatment response and prognosis in 230 patients with primary TNBC who underwent surgery without preoperative chemotherapy between January 2004 and December 2014.

A combination strategy of a semisynthetic macrolide, 5-O-mycaminosyltylonolide with polymyxin B nonapeptide for multi-drug resistance P. aeruginosa.

The emergence and spread of antimicrobial resistant pathogens continue to threaten our ability to combat several infections. Among them, Pseudomonas aeruginosa (P. aeruginosa) poses a major threat to human health.

Rediscovery of Tetronomycin as a Broad-Spectrum and Potent Antibiotic against Drug-Resistant Gram-Positive Bacteria.

Tetronomycin (), first isolated from a cultured broth of sp. by Juslen . in 1974, is a polycyclic polyether compound.

[Successful delivery using interferon α for molecular relapse of chronic myeloid leukemia after interruption of tyrosine kinase inhibitor].

A tyrosine kinase inhibitor (TKI) was used to treat the patient, a 35-year-old woman who was diagnosed with chronic myeloid leukemia at the age of 22 years. Since a four-year deep molecular response (DMR) was obtained, spontaneous pregnancy was planned under TKI withdrawal. Even though her disease had advanced to MR2.

An efflux pump deletion mutant enabling the discovery of a macrolide as an overlooked anti-P. aeruginosa active compound.

Antimicrobial resistance is a serious, worldwide problem. Pseudomonas aeruginosa (P. aeruginosa) is the pathogen that poses a major threat to human health.

Benefits of plerixafor for mobilization of peripheral blood stem cells prior to autologous transplantation: a dual-center retrospective cohort study.

Background Aims: Before autologous stem cell transplantation (ASCT), hematopoietic stem cells must be stimulated to move from the bone marrow to the peripheral blood for harvesting. Plerixafor, a C-X-C chemokine receptor type 4 antagonist, is used to increase stem cell harvests. However, the effects of plerixafor on post-ASCT outcomes remain unclear.

Transient decrease in the depth of the negative T wave in apical hypertrophic cardiomyopathy is a sign of left anterior descending artery stenosis: a case series.

Background: In patients with apical hypertrophic cardiomyopathy (HCM), electrocardiography (ECG) often shows left ventricular hypertrophy (LVH) and a negative T wave. A negative T wave often disappears over time due to degeneration of the apical myocardium. However, there are limited reports on the temporary change of a negative T wave in patients with HCM.

Biomarkers for predicting response to corticosteroid therapy for immune thrombocytopenic purpura.

Patients with immune thrombocytopenic purpura (ITP) often receive corticosteroids as a first-line treatment strategy. The ability to predict the therapeutic response to corticosteroids before initiating treatment would reduce the risk of adverse events, but biomarkers of this parameter have not yet been established. Here, in a single-centre, retrospective, cohort study of 127 ITP patients who received corticosteroids as first-line treatment, we compared several characteristics and test results between those patients with a favourable response to corticosteroids (responder cohort, n = 68) and those with a poor response to corticosteroids (non-responder cohort, n = 59) to identify potential biomarkers that were predictive of corticosteroid response.

Gamma-secretase inhibitor does not induce cytotoxicity in adult T-cell leukemia cell lines despite NOTCH1 expression.

Background: Activated mutations in NOTCH1 are drivers of T-cell type acute lymphoblastic leukemia/lymphoma. The γ-secretase inhibitor (GSI), which suppresses the function of NOTCH1, is expected to be a molecular-targeted agent. NOTCH1 is also expressed in other malignant neoplasms.