UHRF proteins catalyze the ubiquitination of target proteins and are involved in regulating gene expression. Some studies reported a reduced expression of UHRF2 in acute leukemia cells, but the role of UHRF2 in hematopoiesis remains unknown. Here, we generated mice to clarify the role of deletion in hematopoiesis.
View Article and Find Full Text PDFChronic myeloid leukemia (CML) is a myeloproliferative neoplasm driven by the BCR::ABL1 tyrosine kinase. Tyrosine kinase inhibitors (TKIs) have been established as standard therapies for CML. However, some CML patients experience TKI intolerance.
View Article and Find Full Text PDFRavulizumab is an anti-C5 antibody approved for treating paroxysmal nocturnal hemoglobinuria (PNH). In August 2019, a 77-year-old Japanese man with PNH, who had been on ravulizumab treatment for 2 years, was hospitalized for chest discomfort and malaise. Electrocardiography identified a right bundle block, and elevated serum troponin I and d-dimer suggested ischemic heart disease.
View Article and Find Full Text PDFThe pathogenesis of sepsis-induced acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) has not yet been fully elucidated. Growth arrest-specific 6 (Gas6) has marked effects on hemostasis and reduces inflammation through its interaction with receptor tyrosine kinases of the TAM family: Tyro3, Axl, and Mer. Here, we found that plasma concentrations of Gas6 and soluble Mer were greater in patients with severe sepsis or septic ALI/ARDS compared with those in normal healthy donors.
View Article and Find Full Text PDFWe here report a 21-year-old male who presented with acute myelomonocytic leukemia (AMML) associated with acquired von Willebrand syndrome (AVWS). To our knowledge, this is the first case of AVWS caused by AMML. In our case, following remission-induction chemotherapy combined with idarubicin and cytarabine, the patient showed remarkable improvement of bleeding symptoms due to AVWS.
View Article and Find Full Text PDFA 49-year-old man was hospitalized with infectious mononucleosis-like symptoms, hepatosplenomegaly, and circulating abnormal lymphocytes. Hepatosplenic T-cell lymphoma was suspected, and bone marrow aspiration and liver biopsy were performed. The pathological diagnosis was T-cell lymphoma.
View Article and Find Full Text PDFA 76-year-old man presented with a tracheal tumor associated with severe respiratory obstruction. A tracheotomy was performed due to respiratory failure. F-fluorodeoxyglucose (FDG) -positron emission tomography/computed tomography revealed an abnormal accumulation of FDG (maximum standardized uptake value: 16) in the trachea.
View Article and Find Full Text PDFLight chain deposition disease (LCDD) is a rare systemic disorder caused by the deposition of light chain immunoglobulins, which often results in renal impairment associated with either nephrotic syndrome or asymptomatic proteinuria. B-cell neoplasms, such as multiple myeloma and lymphoproliferative disorders, are well-known underlying diseases in LCDD. Some chemotherapy regimens have been reported, but both evidence-based treatment and management for LCDD have yet to be established.
View Article and Find Full Text PDFHigh-mobility group AT-hook 2 () is crucial for the self-renewal of fetal hematopoietic stem cells (HSCs) but is downregulated in adult HSCs via repression by and the polycomb-recessive complex 2 (PRC2) including EZH2. The messenger RNA (mRNA) level is often elevated in patients with myelofibrosis that exhibits an advanced myeloproliferative neoplasm (MPN) subtype, and deletion of promotes the progression of severe myelofibrosis in mice with upregulation of several oncogenes such as . However, the direct role of in the pathogenesis of MPNs remains unknown.
View Article and Find Full Text PDFLess than 5% of patients with polycythemia vera (PV) show JAK2 exon 12 mutations. Although PV patients with JAK2 exon 12 mutations are known to develop post-PV myelofibrosis (MF) as well as PV with JAK2V617F, the role of JAK inhibitors in post-PV MF patients with JAK2 exon 12 mutations remains unknown. We describe how treatment with a JAK1/2 inhibitor, ruxolitinib, led to the rapid amelioration of marrow fibrosis, erythrocytosis and thrombocytopenia in a 77-year-old man with post-PV MF who carried a JAK2 exon 12 mutation (JAK2H538QK539L).
View Article and Find Full Text PDFInt J Hematol
November 2017
We here report a 47-year-old female with autoimmune myelofibrosis (AIMF) associated with liver damage caused by autoimmune hepatitis and Evans syndrome. Bone marrow biopsy revealed hypocellular marrow with grade 2 reticulin fibrosis and increased levels of B lymphocytes (CD20), T lymphocytes (CD3, CD8), and plasma cells (CD138). Immunohistochemical analysis revealed increased expression of transforming growth factor-β (TGF-β) in infiltrating lymphocytes and macrophages in the bone marrow.
View Article and Find Full Text PDFBackground: After acute infection of Epstein-Barr virus, Epstein-Barr virus-infected B cells survive but usually do not show clonal proliferation. However, Epstein-Barr virus-infected B cells occasionally acquire a proliferative capacity that provokes clonal lymphoproliferative disorders. We herein present a case with Epstein-Barr virus-infected CD30+ B cell and immunoglobulin G4+ plasmacytoid cell proliferation in the lymph nodes, suggesting a pathological and clinical interaction between Epstein-Barr virus-associated B-cell lymphoproliferative disorders and immunoglobulin G4-related disease.
View Article and Find Full Text PDFAm J Physiol Regul Integr Comp Physiol
July 2014
Two types of aquaporin 5 (AQP5) genes (aqp-xt5a and aqp-xt5b) were identified in the genome of Xenopus tropicalis by synteny comparison and molecular phylogenetic analysis. When the frogs were in water, AQP-xt5a mRNA was expressed in the skin and urinary bladder. The expression of AQP-xt5a mRNA was significantly increased in dehydrated frogs.
View Article and Find Full Text PDFCK-MB is an important marker for the diagnosis of acute myocardial infarction (AMI). Since mitochondrial CK (MtCK) is universally present in the blood of healthy individuals, it is known to positively affect the measurement of CK-MB using the immunoinhibition method, causing false-positive results. We performed basic evaluation of ACCURAS AUTO CK-MB MtO, a new reagent containing anti-MtCK antibody that inhibits MtCK activity, and attempted to calculate a cut-off CK-MB level to diagnose AMI.
View Article and Find Full Text PDFA 68-year-old woman with recurrent advanced small cell lung cancer (SCLC), previously treated with 7 courses of carboplatin + etoposide, 4 courses of cisplatin + irinotecan and radiotherapy (primary site and whole brain irradiation), received 3 courses of a single nogitecan hydrochloride i.v. bolus with 4 consecutive days of administration, for a total dose of 7.
View Article and Find Full Text PDFTaxane-based chemotherapy now plays an important role in the first-line treatment on patients with advanced non-small-cell lung cancer (NSCLC). Recent attention has been focused on the treatment of patients with NSCLC who failed to respond to taxane-based chemotherapy. In the present article, we report the effect of single-agent vinorelbine (VNR) in 10 patients with NSCLC previously treated with taxanes.
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