[Rinshō ketsueki] The Japanese journal of clinical hematology
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Adult T-cell leukemia-lymphoma (ATL) caused by human T-lymphotropic virus type 1 (HTLV-1) is generally associated with poor prognosis. The anti-CCR4 antibody mogamulizumab is one of the options for refractory or relapsed ATL. Mogamulizumab is intravenously administered as a single agent at a dose of 1.0 mg/kg once a week for 8 weeks. ⋯ HAM is an inflammatory disease of the central nervous system, which is characterized by slowly progressive spastic paresis of bilateral lower limbs and bladder-rectal disorder. Coexistence of ATL and HAM has rarely been reported. Here we present the case of a patient with ATL in whom Takotsubo cardiomyopathy and HAM developed after mogamulizumab administration.
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In the treatment of hemophilia patients, factor (F) VIII or FIX product prophylaxis results in arthropathy prevention and quality of life (QOL) improvement. Serious issues concerning hemostatic treatment of hemophilia include frequent intravenous administration of products, inhibitor development, and hemostatic treatment of patients with inhibitors. To overcome these challenges, products with extended half-life were developed. ⋯ Two therapies, namely si-RNA anti-antithrombin therapy and anti-TFPI antibody therapy, aimed at rebalancing coagulation, are also under development. In addition, a gene therapy for hemophilia B has been developed by improving the vector. These new therapies could further improve the QOL of hemophilia patients.
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Although Hodgkin lymphoma is a disease with a low incidence rate, there is a significant social resources perspective associated with this disease to ensure that adolescents and young adults are adequately treated and receive social reintegration. Consequently, Hodgkin lymphoma is considered an important disease in the field of oncology. Combined modality therapy, which combines chemotherapy and radiotherapy, has resulted in cure rates of more than 80% in patients with early stages of this disease, although long-term sequelae remain a problem. ⋯ Both high cure rates and toxicity control are essential targets for treatment modalities of Hodgkin lymphoma. Long-term follow-up of secondary malignancy and heart and lung failure should be incorporated into such treatment strategies. In this review, I explore comprehensive treatment strategies for Hodgkin lymphoma in the early stage with both favorable and unfavorable prognosis, in the advanced stage, and relapsed or refractory disease.
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Immune checkpoint inhibitors are the most striking innovation in the clinical development of immunotherapy. Monoclonal antibodies (mAbs) restore and augment the antitumor immune activities of cytotoxic T cells by mainly blocking immune checkpoint molecules on T cells or their ligands on antigen-presenting and tumor cells. Based on preclinical data, many clinical trials have demonstrated the acceptable safety profiles and efficacies of mAb in various cancers. ⋯ Current advances in tumor immunology have unveiled the importance of immunosuppressive cells, such as regulatory T cells, myeloid-derived suppressor cells, and tumor-associated macrophages, especially in a tumor microenvironment (TME). Some data from basic research in mouse models and the immunomonitoring of cancer patients suggest that the inhibition of immunosuppressive cells and the cytokines related to them activate and infiltrate cytotoxic T cells and in TME, which could be one of the next combination strategies. The current clinical development of, translational research on, and future challenges in utilizing immune checkpoint inhibitors are described.
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Fournier's gangrene (FG) is a fulminant infective necrotizing fasciitis, which includes the genital, perineal, and perianal regions. A 77-year-old man had previously been diagnosed as having diabetes mellitus (DM) and was treated with pioglitazone (15 mg) and miglitol (150 mg). He developed sudden perineal discomfort, fever with painful penile, and scrotal edema, subsequently leading to urinary retention. ⋯ Bone marrow aspiration revealed hypercellularity with 9% myeloblasts, micromegakaryocytes, abnormal leukocyte granulation, and erythrocytic dyspoiesis, leading to a diagnosis of myelodysplastic syndrome (MDS) RAEB-1, and he was evaluated as high risk according to IPSS-R. After 4 courses of azacitidine treatment, he achieved HI-E and had no further recurrence of FG for more than 18 months. Although DM and alcohol misuse are common systemic comorbidities in patients with FG, MDS should be considered in elderly FG cases, even when DM complications are present.