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Case report: Successful treatment of a patient presenting with a very rare association of acute lymphoblastic leukemia and mucopolysaccharidosis type IVA.

Treating Acute Lymphoblastic Leukemia (ALL) in patients with genetic disorders poses significant challenges for onco-hematologists. Mucopolysaccharidosis type IVA (MPS-IVA) is a lysosomal storage disorder that clinically manifests with progressive and multi-systemic comorbidities, primarily affecting the bone, cartilage, spine, heart and lungs. We report a unique case of B-lineage ALL in a patient with MPS-IVA, who was successfully cured with a personalized chemo-radiotherapy approach. The treatment strategy aimed to balance a curative-intent chemotherapy attempt with the minimization of life-

Patient-Reported Oral Symptoms and Their Impact on Well-Being After Haematopoietic Cell Transplantation.

OBJECTIVE: Oral complications may negatively influence outcomes of haematopoietic cell transplantation (HCT). A comprehensive view of oral symptoms and symptom burden post-HCT is lacking. This study aimed to determine the prevalence, severity, and temporal relationships of oral symptoms and their impact on well-being in the early post-HCT phase. Effects of transplant type and conditioning intensity were evaluated. METHODS: In this prospective multicentre observational study, adult HCT recipients were interviewed and completed questionnaires on oral symptoms and well-being three times a week d

Photobiomodulation in Medication-Related Osteonecrosis of the Jaw: Outcomes in Stage I and Its Adjunctive Role in Advanced Cases.

Background/Objectives: The development of pharmacotherapy, particularly in antiangiogenic drugs, has led to the emergence of MRONJ as a significant side effect. With the increasing incidence of cancer, the management of MRONJ poses a growing challenge for clinicians. The aim of the study is to evaluate the effectiveness of photobiomodulation (PBM) in treating patients with stage I, II, and III medication-related osteonecrosis of the jaw (MRONJ). Methods: A total of 31 patients were divided into two groups: Group 1 (n = 14 patients), with Stage 1 MRONJ; and Group 2 (n = 17 patients), with Stage

Osteonecrosis of the Jaw and Antiresorptive Agents in Benign and Malignant Diseases: A Critical Review Organized by the ECTS.

CONTEXT: Antiresorptive therapy significantly reduces fracture risk in patients with benign bone disease and skeletal-related events (SREs) in patients with bone metastases (BM). Osteonecrosis of the jaw (ONJ) is a rare but severe condition manifested as necrotic bone lesion or lesions of the jaws. ONJ has been linked to the use of potent antiresorptive agents, termed medication-related ONJ (MRONJ). OBJECTIVE: We aimed to identify the differences various aspects of MRONJ among distinct patient categories and provide recommendations on how to mitigate the risk and optimally manage MRONJ in eac

Grading of minor salivary gland immuno-histopathology post-allogenic hematopoietic cell transplantation.

The oral cavity commonly displays mucosal lichenoid lesions and salivary gland dysfunction, which are considered different chronic Graft-versus-Host Disease (cGVHD) pathophysiology's. However, diagnostics of salivary gland (sg-)cGVHD are limited. The objectives of the current study are to evaluate the minor salivary gland (MSG) histo-immunopathological profiles post allogenic hematopoietic cell transplantation based on sg-cGVHD criteria. Design: Histopathology was characterized according to two published grading strategies. Firstly, the National Institute of Health (NIH) assessed peri-ductal/a

Приобретенный изолированный фактор VII Недостаток в ячейке Плазмы: краткое представление Two Дела, связанные с Плазмой-Келл-Леукемией, и обзор литературы.

Приобретенный изолированный фактор VII (FVII) недостаток является редким, но важным открытием у пациентов с расстройствами плазменных клеток, имеющими значительные терапевтические и прогнозные последствия. Цель настоящего анализа и обзора случаев, о которых сообщается в литературе, состоит в том, чтобы высветить связанные с болезнями характеристики, связанные с этим редким свертываемым дефектом, клиническими проявлениями и результатами, а также потенциальными основополагающими механизмами и дать руководящие указания в отношении методов лечения этих пациентов с точки зрения профилактических и т

Novel Developments in the Treatment of Multiple Myeloma-Associated Bone Disease.

Osteolytic bone disease is present in about 80% of patients with multiple myeloma at the time of diagnosis. Managing bone disease in patients with multiple myeloma is a challenge and requires a multi-faceted treatment approach with medication, surgery, and radiation. The established treatments with intravenous or subcutaneous antiresorptives can cause debilitating adverse events for patients, mainly osteonecrosis of the jaw, which, traditionally, has been difficult to manage. Now, oral surgery is recommended and proven successful in 60-85% of patients. Patients with spinal involvement may bene

Multiple Myeloma Associated Bone Disease.

The lytic bone disease is a hallmark of multiple myeloma, being present in about 80% of patients with newly diagnosed MM, and in more during the disease course. The myeloma associated bone disease (MBD) severely affects the morbidity and quality of life of the patients. MBD defines treatment demanding MM. In recent years, knowledge of the underlying pathophysiology has increased, and novel imaging technologies, medical and non-pharmaceutical treatments have improved. In this review, we highlight the major achievements in understanding, diagnosing and treating MBD. For diagnosing MBD, low-dose

Clinical efficacy analysis of mucoperiosteal flap combined with perforation technique for tooth extraction in medication-related osteonecrosis of the jaw risk population: a retrospective study.

BACKGROUND: To analyze and summarize the clinical efficacy of mucoperiosteal flap combined with perforation technique for tooth extraction in medication-related osteonecrosis of the jaw risk (MRONJ) population, and to provide clinical treatment options for such patients. METHODS: This study included 51 patients receiving antiresorptive drug (ARD) treatment who underwent tooth extraction at the Department of Oral and Maxillofacial Surgery, Peking University School and Hospital of Stomatology between November 2016 and February 2024. Preoperative clinical data were collected and analyzed. Based

Application of Autologous Platelet-Rich Plasma on Tooth Extraction Site Prevents Occurence of Medication-Related Osteonecrosis of the Jaws in Rats.

This study evaluated the effects of local application of autologous platelet-rich plasma (PRP) on the tooth extraction site of rats presenting the main risk factors for medication-related osteonecrosis of the jaw (MRONJ). For seven weeks, senile rats were submitted to systemic treatment with vehicle (VEH and VEH-PRP) or 100 μg/Kg of zoledronate (ZOL and ZOL-PRP) every three days. After three weeks, the first lower molar was extracted. VEH-PRP and ZOL-PRP received PRP at the tooth extraction site. Euthanasia was performed at 28 days postoperatively. Clinical, histopathological, histometric and

Salivary Osteopontin as a Potential Biomarker for Oral Mucositis.

Osteopontin (OPN), a multifunctional phosphoglycoprotein also presents in saliva, plays a crucial role in tumour progression, inflammation and mucosal protection. Mucosal barrier injury due to high-dose conditioning regimen administered during autologous and allogeneic peripheral stem cell transplantation (APSCT) has neither efficient therapy nor established biomarkers. Our aim was to assess the biomarker role of OPN during APSCT, with primary focus on oral mucositis (OM). Serum and salivary OPN levels were determined by ELISA in 10 patients during APSCT at four stages of transplantation (day

From Pathogenesis to Intervention: The Importance of the Microbiome in Oral Mucositis.

Oral mucositis (OM) is a common and impactful toxicity of standard cancer therapy, affecting up to 80% of patients. Its aetiology centres on the initial destruction of epithelial cells and the increase in inflammatory signals. These changes in the oral mucosa create a hostile environment for resident microbes, with oral infections co-occurring with OM, especially at sites of ulceration. Increasing evidence suggests that oral microbiome changes occur beyond opportunistic infection, with a growing appreciation for the potential role of the microbiome in OM development and severity. This review c

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… The impact of timing and modalities of dental prophylaxis on the risk of 5-fluorouracil-related oral mucositis in patients with head and neck cancer: A nationwide population-based cohort study Support. Care Cancer 2021 29 3163 3171 10.1007/s00520-020-05825-y 33074358 76. Saunders D.P. Rouleau T. …

Fluoroquinolone Prophylaxis Uncovers High Prevalence Rates of Fluoroquinolone-Resistant Enterobacterales Colonization in Multiple Myeloma Autologous Transplant Patients: A Prospective Cohort Study.

Background: Fluoroquinolone prophylaxis during autologous stem cell transplantation (aSCT) reduces the risk of fever but raises the risk of bloodstream infection (BSI) with fluoroquinolone-resistant Enterobacterales (FRE). We performed a prospective cohort study to detect the presence and potential gain or loss of colonic FRE colonization using serial sampling before and after aSCT in a uniform population of patients with a diagnosis of multiple myeloma. Methods: Eligible subjects underwent aSCT after conditioning with dose-intense melphalan, 200 mg/m2. Peri-anal swabs were obtained before aSC

Мелатонин за пределами хронобиологии: интеграция фармакокинетики, прогнозирование дозы и клинические данные.

Мелатонин установлен в качестве регулятора времени и сна в циркадиане, в то время как более широкий спектр зависящих от рецептора и независимых от рецептора биологических эффектов вызвал интерес к дополнительным видам терапевтического применения. В этом обзоре критически рассматривается вопрос о том, можно ли интерпретировать эти предлагаемые последствия в фармакологических рамках, основанных на воздействии. Мы интегрируем недавние доклинические и клинические данные с фармакокинетиками перорального и внутривенного мелатонина и с обычным дифференциальном уравнением (ОДВ) - прогнозом воздействия

Evaluation of the Incidence and Stage of Oral Mucositis in Patients Undergoing Hematopoietic Stem Cell Transplantation: A Retrospective Study.

AimThis study aims to retrospectively evaluate the incidence and stage of oral mucositis in patients undergoing hematopoietic stem cell transplantation.MethodsA total of 102 patient records of patients hospitalized between 2014 and 2019 in the adult hematopoietic stem cell transplantation clinic of a tertiary university hospital in Turkey were evaluated. Data were collected through a retrospective evaluation of patient records. Records made according to the WHO Oral Toxicity Scale included in the patient records during hospitalization in the adult hematopoietic stem cell transplantation clinic

The Influence of Local Pamidronate Application on Alveolar Dimensional Preservation after Tooth Extraction-An Animal Experimental Study.

The aim of this randomized, controlled animal exploratory trial was to investigate the influence of local application of aminobisphosphonate pamidronate during the socket preservation procedure. Mandibular premolars were extracted in five Göttingen minipigs. Two animals underwent socket preservation using BEGO OSS (n = 8 sockets) and three animals using BEGO OSS + Pamifos (15 mg) (n = 12 sockets). After jaw impression, cast models (baseline, eight weeks postoperative) were digitized using an inLab X5 scanner (Dentsply Sirona) and the generated STL data were superimposed and analyzed with GOM I

Female sex as an independent prognostic factor in the development of oral mucositis during autologous peripheral stem cell transplantation.

Oral mucositis (OM) is a frequent complication of stem cell transplantation-associated toxicity in haematological malignancies, contributing to mortality. Therapy still remains mainly supportive. We assessed risk factors in retrospective analysis of 192 autologous peripheral stem cell transplantation patients with lymphoma and multiple myeloma (MM), respectively. Futhermore, we examined the hormone levels both in serum and saliva during transplantation in 7 postmenopausal female patients with lymphoma compared to healthy controls using electrochemiluminescence immunoassay (ECLIA). Multivariabl

Bisphosphonate- and disumab-related gingival disorders: case analysis from the U.S. Food and Drug Administration Adverse Event Reporting System.

Prior research has indicated that bisphosphonates (BPs) can improve periodontal disease because of their anti-osteoporosis properties. In vitro studies have shown that BPs induce cytotoxicity, inhibit wound healing, and thus affect periodontal disease. Denosumab and BPs have alternative indications. BP and denosumab are not known to correlate with gingival disorders. We assessed such a relationship by applying Bayesian and nonproportional analyses to data in the US FDA Adverse Event Reporting System (FAERS) database. The study analyzed BPs and denosumab-reported incidents with preferred terms

Multidisciplinary Management of Morbidities Associated with Chronic Graft-Versus-Host Disease.

Chronic graft-versus-host disease (cGVHD) represents a common long-term complication after allogeneic hematopoietic stem cell transplantation (HSCT). It imposes a significant morbidity burden and is the leading cause of non-relapse mortality among long-term HSCT survivors. cGVHD can manifest in nearly any organ, severely affecting the quality of life of a transplant survivor. While the mainstay of treatment has remained systemic immunosuppression with glucocorticoids, progress has been made within the last few years with approvals of three oral agents to treat steroid-refractory cGVHD: ibrutin

Multidisciplinary supportive care in systemic light chain amyloidosis.

The immunoglobulin light-chain amyloidosis is a multisystemic disease which manifests by damage to the vital organs by light chain-derived amyloid fibril. Traditionally, the treatment has been directed to the underlying plasma cell clone with or without high dose chemotherapy followed by autologous stem cell transplantation using melphalan based conditioning. Now with the approval of highly tolerable anti-CD38 monoclonal antibody daratumumab based anti-plasma cell therapy in 2021, high rates of hematologic complete responses are possible even in patients who are otherwise deemed not a candidat