A rare case of oral multisystem Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by high proliferation of Langerhans dendritic cells. LCH is a solitary or multifocal disease that primarily involves bone tissue and often affects children and young men. A 29 years-old Caucasian man was referred to the Oral Surgery Unit of George Eastman Hospital - Umberto I teaching hospital, with third degree mobility of teeth belonging to second, third and fourth quadrant. Panoramic radiograph showed multiple radiolucent areas with well demarcated borders on the right and left site of the mandible and on the left site of the maxilla. Extractions of compromised teeth and biopsy of the osteolytic tissue were performed. The final diagnosis of multisystem Langerhans cell histiocytosis of the soft and hard tissues of the oral cavity was made. The patient was sent to the Hematology department of Umberto I Teaching Hospital of ?Sapienza? ? University of Rome for the proper treatment. The present case of rare multisystem LCH involving oral hard and soft tissues shows the strong importance of better investigate, with appropriate additional exams, initial shifty symptoms that could lead to a misdiagnosis

DIAGNOSTIC ACCURACY OF BONE SCINTIGRAPHY IN THE EARLY PREDICTION OF MRONJ

Since Medication-Related Osteonecrosis of the Jaw (MRONJ) is challenging to treat, prevention and early detection are fundamental to limit progression. While some imaging techniques such as computed tomography (CT) scans are helpful to estimate the extent of osteonecrosis in clinically evident MRONJ, methods for early detection and identification of patients at risk for MRONJ need further investigations. The aim of our retrospective study was to evaluate the role of bone scintigraphy (BS) in the early prediction of MRONJ. BS of patients treated with antiangiogenic or antiresorptive therapy for bone metastases were evaluated for pathological tracer uptake of the jaws. Sensitivity, specificity, predictive values of BS for the prediction of MRONJ were investigated. Moreover, the latency from the first presence of pathological tracer uptake in BS to clinically evident MRONJ was determined. The significance of pathologic BS for MRONJ detection was calculated by Fisher's exact test and the odds ratio was determined.A total of 19 patients with representative BS were recruited from our Department database. Whole body BS in anterior and posterior positions were reviewed by a specialist in nuclear medicine, blinded to MRONJ diagnosis, for pathological tracer uptake of the jaws. Results were compared to development of clinically evident MRONJ. Sensitivity and specificity of BS for MRONJ prediction were respectively 90% and 82%. Positive and negative predictive values were 64% and 96%, respectively. Median time from the first presence of pathological tracer uptake in BS to clinically evident MRONJ was 20.3 months (range 7–41). Pathologic findings on BS were significantly (p < 0.001) more often observed in patients who developed MRONJ compared to patients who did not. The risk to develop MRONJ was 41.4-fold increased when BS was pathologic. According to our results, BS is an accurate method for the prediction of MRONJ

Clinical aspects of bisphosphonate-associated oral osteonecrosis in patients with multiple myeloma

Background. Bisphosphonates are drugs which act on bone metabolism. They act on osteoclastic activity and inhibit neoangiogenesis. In the bisphosphonate class the most clinically used molecules are pamidronate and zolendronate. They represent “the gold standard” and are used in multiple myeloma treatment, solid tumors with bone metastases, and prevention of pathological fractures. In recent years it has been supposed that bisphosphonates may lead to jaws osteonecrosis, expecially after dentistry procedures such as dental extractions, implant surgery and periodontics interventions. Our work aim is to delineate clinical aspects of patients’ lesions come to our observation for jaws osteonecrosis. These belonged to a patients’ group treated by bisphosphonates for multiple myeloma, osteoporosis, hyperparathyroidism, Paget’s disease. Patients and methods. We examinated clinical data of 9 patients(8 females, 1 male), with jaws osteonecrotic lesions and treated by bisphosphonates, for a mean time of 42 months. Six of the 9 patients were treated for multiple myeloma. In 4 cases osteonecrotic were located on superior maxillary, in 5 ones on jaw. Clinically exposed bone area was infected, with radiated and burning pain, and antibiotic therapy was necessary. Results. Most clinically represented lesion was an area of ulcerated mucosa with not vital exposed bone. Flogosis was typical in area surrounding the necrosis. Neither bleeding nor pain could be appreciated in the exposed bone area. Painful lesions make more difficult oral hygiene, with frequent onset of local parodontosis. At radiographic exams typical illness marks are not corresponded instead. A standardized protocol for jaws osteonecrosis doesn’t exist. In our clinical trial we performed several clinical plans, with different results. The suspension of bisphosphonate therapy does not appear to hasten recovery of the osteonecrosis. Despite antibiotic therapy, resective surgery, osteoplastics and hyperbaric oxygen therapy most of the lesions did not respond well to therapy. Discussion. Although bisphosponates use started about 30 years ago, first osteonecrotic lesions were described in 2003 (Max and Stern). The pathogenesis is correlated to the modifications of vascularization induced from such drugs, but would compete even factors as masticatory stress, iatrogenic procedures, local bacterial position, association with other rugs and some systemic pathologies. Surgical treatment may lead to not predictable results and recurrences. Antibiotic therapy reduces the inflammation and the pain, but did not assure healing; therapies with disinfectant and irrigantes have not produced results; in our clinic the hyperbaric oxygen therapy has gotten good results toward in the comparisons of the inflammation and in the improvement of the course-operating after sequestrotomy. Conclusions. Bisphosphonates are essential drugs in the treatment of several diseases other than osteoporosis. But considering the impact on the quality of life of the patients on which rise up osteonecrosis of the maxillary, it is necessary to perform odontostomatologic visit before the therapy, and to evaluated the relationship between benefit of the treatment and the risk of such lesion

A SINGLE REFERENCE CENTER, 2 YEARS RETROSPECTIVE STUDY ON THE RISK FACTORS ASSOCIATED WITH ANTI-RESORPTIVE MEDICATION-RELATED OSTEONECROSIS OF THE JAWS IN OSTEOPOROTIC AND ONCOLOGICAL PATIENTS

Medication-related osteonecrosis of the jaws (MRONJ) has multiple exogenous and endogenous risk factors. The risk of MRONJ associated with anti-resorptive drugs (Bisphosphonates and Denosumab) was reviewed in oncological and osteoporotic patients referred to the Department of Oral and Maxillo-Facial Sciences, Sapienza University of Rome (Italy), since January 2019 to January 2021. The incidence of MRONJ was analyzed according to local (dental extraction), systemic risk factors (diabetes, steroid, ecc), medication type, and drug treatment duration. The intake of Zolendronate was strongly associated with osteonecrosis of the jaws in oncological patients and the risk of MRONJ was higher in patients with metastases regardless of the type of anti-resorptive therapy. The use of Bisphosphonates and Denosumab was very common in the treatment of osteoporosis, up to 42.4% of the patients, and the incidence of MRONJ was higher than previously reported. It was possible to prevent the occurrence of MRONJ after oral surgery due to medical prophylaxis, non-traumatic surgical procedures, and photobiomodulation therapy (PBMT)

Clinical practice of personalized prophylaxis in hemophilia: Illustrations of experiences and benefits from two continents.

These cases of people with hemophilia (PWH) illustrate the importance of understanding the patient's expectations and desires and adapting treatment to meet these needs, in addition to traditional clinical targets. Population PK modeling and FVIII products with improved PK profiles provide the opportunity to individualize care and improve long-term outcomes

Management of Medication-Related Osteonecrosis of the Jaw (MRONJ) Using Leukocyte- and Platelet-Rich Fibrin (L-PRF) and Photobiomodulation: A Retrospective Study

Background. The aim of this study was to compare retrospectively the effect of three different treatment protocols on the healing outcome in patients with established medication-related osteonecrosis of the jaw (MRONJ). Methods. A total of 34 MRONJ patients were recruited from the Department database and were divided according to the treatment protocols in a study group (G1) and two control groups (G2 and G3). G1 was treated with antibiotic therapy, surgery, leukocyte- and platelet-rich fibrin (L-PRF), and photobiomodulation; G2 was treated with antibiotic therapy and surgery; G3 was treated with antibiotic therapy and photobiomodulation. Various clinical variables and treatment protocols were analyzed to determine their correlation with the healing outcome at three and six months of follow-up. Results. There was a significant association between the different treatment protocols and the outcomes at both three and six months follow-up (p = 0.001 and p = 0.002, respectively). No significant association was observed between the outcomes and MRONJ localization, MRONJ stage, duration of drug treatment, gender, diabetes, corticosteroid therapy, smoking habits, underlying disease, and history of chemotherapy at both three and six months follow-up. Conclusions. Our results show that the combination of antibiotic therapy, surgery, L-PRF, and photobiomodulation may effectively contribute to MRONJ management