ABSTRACT
Hematopoietic stem cell transplants for inherited metabolic disorders performed at Tokai University Hospital between June 5, 1986, and May 28, 2021, were analyzed and compared between the period before 2007 and the period from 2007 onward based on availability of medical resources. Transplants were performed for 38 patients with mucopolysaccharidosis, 33 with adrenoleukodystrophy, and 16 with another disorder. Before 2007, oral busulfan-based regimens were mainly used. From 2007 onward, intravenous busulfan-based regimens or 4 Gy of thoracoabdominal irradiation (TAI), fludarabine, and melphalan (Mel)/treosulfan were adopted. Between 2002 and 2010, adrenoleukodystrophy was treated with 12 Gy of TAI and Mel. HLA-identical sibling bone marrow was used in 43% of cases before 2007 and 15% from 2007 onward, while alternative donors were selected for other transplants. Overall survival and event-free survival (EFS) before 2007 and from 2007 onward were 76% and 62%, and 97% and 85%, respectively (P = 0.006 and 0.017). Transplant era predicted superior overall survival and EFS, while myeloablative conditioning also predicted EFS. The incidence of primary graft failure decreased from 2007 onward, especially in cord blood transplant when 4 Gy of TAI with 150 mg/m2 fludarabine and 180 mg/m2 Mel or 42 g/m2 treosulfan were used as conditioning.
ABSTRACT
Photochemical multi-component coupling reactions initiated by the activation of glycosyl bromides in the presence of 1,4-bis(diphenylamino)benzene (BDB) as an organic photocatalyst were developed. C-glycosides accompanied by olefin (di)functionalization were obtained. This method allows us to access various C-glycosides with alkene, carbonyl, alcohol, ether, and amide functionalities.
ABSTRACT
Panniculitis is an inflammation that occurs in subcutaneous adipose tissue. Panniculitis includes physical panniculitis (e.g., traumatic) and infectious panniculitis (e.g., bacterial, fungal, subcutaneous panniculitis-like T cell lymphoma [SPCTL], etc.). Accurate diagnosis is crucial due to similar clinical presentation of all types of panniculitis. Here, we report a case of SPCTL which was initially diagnosed with traumatic panniculitis. A 15-year-old male patient was admitted to a previous hospital due to a progressively enlarged right flank and inguinal mass after an abdominal bruise. He was initially diagnosed with traumatic panniculitis, but the mass expanded throughout the chest and abdomen accompanied by a fever of over 11 months. Computed tomography (CT) revealed a subcutaneous mass in the anterior chest and abdominal wall. Fludeoxyglucose F18 (FDG) uptake was observed at those lesions using FDG-positron emission tomography (PET). A biopsy of the mass lesion was performed, during which SPCTL was diagnosed based on pathological examination. He was initially treated with prednisolone and cyclosporine A for two weeks. His fever went down, but subcutaneous mass in the chest and abdominal wall persisted. Therefore, he received a cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) regimen. After 6 courses of CHOP, CT revealed no disease evidence. He remained in complete remission at 30 months of therapy.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols , Cyclophosphamide , Disease Progression , Doxorubicin , Lymphoma, T-Cell , Panniculitis , Vincristine , Humans , Male , Panniculitis/diagnosis , Panniculitis/etiology , Panniculitis/drug therapy , Panniculitis/pathology , Adolescent , Lymphoma, T-Cell/diagnosis , Lymphoma, T-Cell/pathology , Lymphoma, T-Cell/diagnostic imaging , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Cyclophosphamide/administration & dosage , Doxorubicin/administration & dosage , Vincristine/administration & dosage , Prednisone/administration & dosage , Tomography, X-Ray Computed , Positron-Emission Tomography , Fluorodeoxyglucose F18 , Treatment Outcome , Biopsy , Diagnosis, DifferentialABSTRACT
TAFRO syndrome is an inflammatory disorder of unknown etiology characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly. Despite great advancements in research on the TAFRO syndrome in the last decade, its diagnosis and treatment are still challenging for most clinicians because of its rarity and severity. Since the initial proposal of the TAFRO syndrome as a distinct disease entity in 2010, two independent diagnostic criteria have been developed. Although these are different in the concept of whether TAFRO syndrome is a subtype of idiopathic multicentric Castleman disease or not, they are similar except for the magnitude of lymph node histopathology. Because there have been no specific biomarkers, numerous diseases must be ruled out before the diagnosis of TAFRO syndrome is made. The standard of care has not been fully established, but interleukin-6 blockade therapy with siltuximab or tocilizumab and anti-inflammatory therapy with high-dose corticosteroids are the most commonly applied for the treatment of TAFRO syndrome. The other immune suppressive agents or combination cytotoxic chemotherapies are considered for patients who do not respond to the initial treatment. Whereas glowing awareness of this disease improves the clinical outcomes of patients with TAFRO syndrome, further worldwide collaborations are warranted.
ABSTRACT
Oral and maxillofacial trauma is influenced by various factors, including regional characteristics and social background. Due to the coronavirus disease 2019 (COVID-19) pandemic, a state of emergency was declared in Japan in March 2020. In this study, we aimed to examine the dynamics of patients with oral and maxillofacial trauma over a 12-years period using interrupted time-series (ITS) analysis. Patients were examined at the Shimane University Hospital, Maxillofacial Trauma Center from April 2012 to April 2023. In addition to general patient characteristics, data regarding the type of trauma and its treatment were obtained from 1203 patients (770 men and 433 women). Group comparisons showed significant differences in age, trauma status, method of treatment, referral source, route, and injury occasion. ITS analysis indicated significant changes in combined nasal fractures, non-invasive reduction, and sports injuries (P < 0.05), suggesting COVID-19 significantly impacted oral and maxillofacial trauma dynamics. A pandemic of an infectious disease may decrease the number of minor trauma cases but increase the number of injuries from outdoor activities, resulting in no overall change in the dynamics of the number of trauma patients. Medical systems for oral and maxillofacial trauma should be in place at all times, independent of infectious disease pandemics.
Subject(s)
COVID-19 , Interrupted Time Series Analysis , Maxillofacial Injuries , Humans , COVID-19/epidemiology , Female , Male , Maxillofacial Injuries/epidemiology , Adult , Middle Aged , Aged , Japan/epidemiology , Pandemics , Young Adult , Adolescent , SARS-CoV-2/isolation & purification , Trauma Centers/statistics & numerical data , Child , Aged, 80 and overABSTRACT
BACKGROUND: Peripheral ossifying fibroma is a nonneoplastic inflammatory hyperplasia that originates in the periodontal ligament or periosteum in response to chronic mechanical irritation. Peripheral ossifying fibroma develops more commonly in young females as a solitary, slow-growing, exophytic nodular mass of the gingiva, no more than 2 cm in diameter. While various synonyms have been used to refer to peripheral ossifying fibroma, very similar names have also been applied to neoplastic diseases that are pathologically distinct from peripheral ossifying fibroma, causing considerable nomenclatural confusion. Herein, we report our experience with an unusual giant peripheral ossifying fibroma with a differential diagnostic challenge in distinguishing it from a malignancy. CASE PRESENTATION: A 68-year-old Japanese male was referred to our department with a suspected gingival malignancy presenting with an elastic hard, pedunculated, exophytic mass 60 mm in diameter in the right maxillary gingiva. In addition to computed tomography showing extensive bone destruction in the right maxillary alveolus, positron emission tomography with computed tomography revealed fluorodeoxyglucose hyperaccumulation in the gingival lesion. Although these clinical findings were highly suggestive of malignancy, repeated preoperative biopsies showed no evidence of malignancy. Since even intraoperative frozen histological examination revealed no malignancy, surgical resection was performed in the form of partial maxillectomy for benign disease, followed by thorough curettage of the surrounding granulation tissue and alveolar bone. Histologically, the excised mass consisted primarily of a fibrous component with sparse proliferation of atypical fibroblast-like cells, partly comprising ossification, leading to a final diagnosis of peripheral ossifying fibroma. No relapse was observed at the 10-month follow-up. CONCLUSIONS: The clinical presentation of giant peripheral ossifying fibromas can make the differential diagnosis from malignancy difficult. Proper diagnosis relies on recognition of the characteristic histopathology and identification of the underlying chronic mechanical stimuli, while successful treatment mandates complete excision of the lesion and optimization of oral hygiene. Complicated terminological issues associated with peripheral ossifying fibroma require appropriate interpretation and sufficient awareness of the disease names to avoid diagnostic confusion and provide optimal management.
Subject(s)
Fibroma, Ossifying , Gingival Neoplasms , Humans , Fibroma, Ossifying/surgery , Fibroma, Ossifying/pathology , Fibroma, Ossifying/diagnostic imaging , Male , Aged , Diagnosis, Differential , Gingival Neoplasms/pathology , Gingival Neoplasms/surgery , Gingival Neoplasms/diagnostic imaging , Gingival Neoplasms/diagnosis , Maxillary Neoplasms/pathology , Maxillary Neoplasms/surgery , Maxillary Neoplasms/diagnostic imaging , Tomography, X-Ray Computed , Maxilla/pathology , Maxilla/diagnostic imaging , Maxilla/surgeryABSTRACT
BACKGROUND: As the Japanese population may have less genetic diversity than other ethnic groups, treatment outcomes may be affected when allogeneic hematopoietic cell transplantation is performed in other races. However, evidence explaining the effect of racial differences is limited. METHODS: We used the Japanese National Database to examine the outcomes of first allogeneic bone marrow transplantations (BMTs) performed between Japanese and non-Japanese patients from 1996 to 2021. We performed propensity score matching using sex, age group, underlying disease group, HLA mismatch, conditioning regimen intensity, and BMT implementation age to select Japanese-to-Japanese BMT patients as the controls. RESULTS: The numbers of non-Japanese-to-Japanese and Japanese-to-non-Japanese BMT cases included in the analysis were 48 and 75, respectively, and the following outcomes were compared: overall survival, non-relapse mortality, acute graft-vs-host disease (GVHD) ≥ grade II, chronic GVHD, and engraftment of neutrophils and platelets. Most parameters did not differ when comparing BMTs according to ethnicity; only platelet engraftment was delayed in Japanese-to-non-Japanese BMT but not in non-Japanese-to-Japanese BMT. CONCLUSIONS: The results of this study suggested that BMT performed in Japanese and non-Japanese patients has little effect on treatment outcomes. The results of this study may be useful for donor selection in Japan, where internationalization has progressed in recent years.
Subject(s)
Graft vs Host Disease , Hematopoietic Stem Cell Transplantation , Humans , Bone Marrow Transplantation/adverse effects , Japan , Treatment Outcome , Graft vs Host Disease/epidemiologyABSTRACT
In Japan, the incidence of double cancers is increasing due to the rise in cancer prevalence in the super-aging society, and the frequency is particularly high among patients with head and neck cancer. To the best of our knowledge, there has been only 1 reported case of metachronous double cancer with parotid gland cancer and tongue squamous cell carcinoma, and herein we report the outline of this case. The patient was a 70-year-old male with a smoking history. In 2017, a total parotidectomy and neck dissection for adenocarcinoma of the right parotid gland were performed at the otolaryngology department, followed by concurrent chemoradiotherapy as postoperative therapy. Pathological examination revealed adenocarcinoma NOS(pT4aN2bM0, Stage â £A). There was no recurrence or metastasis, and the patient was recovering well. However, in October 2022, the patient presented to the hospital with a chief complaint of pain at the right margin of the tongue. At the initial consultation, an ulcerative lesion with a slightly unclear boundary and maximum diameter of approximately 15 mm was observed on the right margin of the tongue. Since epithelialization was observed in some regions, it was diagnosed as intractable stomatitis and the patient was put under observation for the time being. With no signs of healing during the follow- up examination a month later and considering the possibility of malignant tumors, a total excision of tongue lesion was performed. Pathological examination revealed well-differentiated squamous cell carcinoma(pT1N0M0, Stage â ). Going forward, we plan to conduct careful follow-up observations with the possibility of further metachronous double cancer occurrences in mind.