Internal medicine
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Currently, immune checkpoint inhibitors (ICIs) are the standard treatment for malignant pleural mesothelioma (MPM). The characteristic responses to ICI treatment include pseudoprogression (PP) and hyperprogressive disease (HPD), which require attention. ⋯ This was initially judged as HPD but was subsequently diagnosed as PP owing to a marked reduction in tumor size. This case highlights the importance and difficulty of differentiating between PP and true progression, including HPD, during treatment of MPM with ICIs.
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Fabry disease (FD) is a rare X-linked lysosomal storage disorder. Enzyme replacement therapies (ERT), such as agalsidase α and β, are available treatment options. ⋯ We report the case of a female patient with Fabry disease who developed unexpected hypotension after six years of stable treatment with agalsidase α, leading to a switch to agalsidase β. Continuous monitoring may be essential to identify potential IRRs in female patients with Fabry disease receiving long-term ERT.
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This is the first report of therapy-related mixed-phenotype acute leukemia (MPAL) characterized by B-cell, T-cell, and myeloid lineage phenotypes. A 68-year-old man, who was in complete remission after chemoradiotherapy for recurrent esophageal cancer, developed bacterial pneumonia with increasing blasts in the peripheral blood, and a subsequent bone marrow biopsy revealed 85.2% blasts. ⋯ After the initiation of venetoclax and azacitidine, the patient achieved complete remission, and cord blood transplantation was performed. This case provides novel insights into the therapeutic options for therapy-related trilineage MPAL treatment.
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Cardiac amyloidosis (CA), predominantly caused by amyloid transthyretin (ATTR) or immunoglobulin light chain (AL), has a poor prognosis, and a precise diagnosis is crucial because the optimal treatments differ between the two conditions. However, diagnosing AL-CA is challenging because of the unavailability of effective AL-detecting antibodies, particularly in cases with coexisting AL- and ATTR-CA. This report presents a successfully diagnosed case of complicated AL- and ATTR-CA that was successfully treated with tafamidis for ATTR-CA and combination chemotherapy for AL-CA. This case highlights the importance of a precise diagnosis of CA and supports the efficacy and safety of concurrent treatment for coexisting amyloidosis.
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Objective The prevalence rates of bronchial asthma (BA) and chronic obstructive pulmonary disease (COPD) are 3%-11% and 8%-16%, respectively, in the general Japanese adult population. Few reports on patients' perceptions of BA, cough variant asthma (CVA), COPD, and asthma and COPD overlap (ACO) are available in Japan, and we aimed to investigate the agreement between the perception and diagnosis of BA and COPD-related diseases. Methods The subjects were 229 datasets matched to the sex and age distribution of the Japanese population of 1,000 adult patients who underwent respiratory function tests and screening for each disease at Nagasaki University Hospital between July 2014 and July 2017. ⋯ Conclusion Self-perception of asthma-related disease was adequately high, whereas that of COPD-related disease was low. The prevalence rates of BA, COPD, ACO, and CVA in our study were 10.0%, 11.8%, 2.6%, and 0.9%, respectively. An increase in the perception of COPD may help improve community healthcare for respiratory diseases.