Cancer in buccal mucosa in patients with Fanconi anemia: Report of six cases.

INTRODUCTION: Fanconi anemia (FA) is a recessive hereditary disease characterized by bone marrow failure, and the treatment is hematopoietic stem cell transplantation (HSCT). Patients diagnosed with FA are more predisposed to develop oral squamous cell carcinoma (SCC), and this risk increases in transplant patients. The clinical characteristics of the oral manifestations of SCC in this group of patients do not differ from the lesions present in patients without the disease; however, they can be diagnosed in young patients and less common locations, such as, for example, in the buccal mucosa. OBJECTIVE: To report a case series of patients diagnosed with FA with oral SCC. METHOD: Included in this case series are six patients diagnosed with SCC in the buccal mucosa with similar clinical characteristics. FINAL CONSIDERATIONS: There are still difficulties in establishing the natural history of oral lesions in patients with FA. Thus, disclosing a series of cases with similar changes may be relevant to improving and refining the multidisciplinary team's clinical view of suspected SCC or oral potentially malignant disorders (OPMD), providing surveillance and timely management.

Atenção à saúde bucal de pacientes com coagulopatias hereditárias / Atención de salud bucal parapacientes con coagulopatías hereditarias: la contribución de los estudiantes a la reestructuración de la atención / Oral health care for patients with hereditary coagulopathies: the students' contribution to the re-structuring of health care

Por apresentarem maior risco de sangramento durante a realização de procedimentos odontológicos, pacientes diagnosticados com coagulopatias ou trombopatias hereditárias apresentam, frequentemente, dificuldade de acesso à assistência odontológica na Atenção Primária em Saúde. Alguns centros especializados no tratamento dessas doenças contam com o cirurgião-dentista como parte da equipe multiprofissional, permitindo que a maioria dos pacientes realizem o acompanhamento odontológico em tais serviços. Este estudo tem como objetivo relatar a experiência dos discentes de graduação e pós-graduação em Odontologia da Universidade Federal do Paraná na reabertura do ambulatório odontológico de assistência à pacientes com coagulopatias e trombopatias hereditárias do estado. Com a aposentadoria da profissional responsável pelos atendimentos odontológicos deste serviço, os pacientes ficaram desassistidos. Para suprir a demanda reprimida, o ambulatório foi reaberto como atividade extramuros da graduação e pós-graduação em Odontologia. Este fato possibilitou, além da retomada ao cuidado odontológicode pacientes com predisposição a hemorragias, o aprimoramento dos alunos no atendimento de pacientes com comprometimento sistêmico. A reabertura de um ambulatório de alta complexidade em saúde como campo de prática acadêmico é atípico, uma vez que comumente o processo se dá de forma inversa: o discente ingressando em um serviço bem estabelecido. Na presente experiência, os discentes tiveram atuação fundamental no restabelecimento da assistência à saúde pelo ambulatório de Odontologia do hemocentro (AU).

Debido a su mayor riesgo de hemorragia durante los procedimientos dentales, los pacientes diagnosticados con coagulopatías o trombopatías hereditarias a menudo tienen dificultades para acceder a la atención dental en la Atención Primaria de Salud. Algunos centros especializados en el tratamiento de estas enfermedades cuentan con el odontólogo como parte del equipo multidisciplinario, haciendo que la mayoría de los pacientes se sometan a un seguimiento odontológico en dichos servicios. Este estudio tiene como objetivo reportar la experiencia de estudiantes de pre y posgrado en Odontología de la Universidade Federal do Paraná en la reapertura de laconsulta externa de odontología para pacientes con coagulopatías y trombopatías hereditarias en el estado. Con la jubilación del profesional responsable de la atención odontológica de este servicio, los pacientes quedaron desatendidos. Para atender la demanda reprimida, se reabrió el ambulatorio como actividad extramuros para los cursos de grado y posgrado en Odontología. Este hecho posibilitó, además de la reanudación de la atención odontológica de los pacientes con predisposición a la hemorragia, la mejora de los estudiantes en la atención de los pacientes con deterioro sistémico. La reapertura de una clínica de salud de alta complejidad como campo de práctica académica es atípica, ya que el proceso comúnmente se da a la inversa: el estudiante ingresa a un servicio bien establecido. En la experiencia actual, los estudiantes tuvieron un papel fundamental en el restablecimiento de la atención de la salud por parte de la consulta externa de Odontología del Hemocentro (AU).

Due to their increased risk of bleeding during dental procedures, patients diagnosed with hereditary coagulopathies or thrombopathies often have difficulty in accessing dental care in primary health care. Some centres specialised in the treatment of these diseases have a dentist as part of the multidisciplinary team, making most patients undergo dental follow-up in such services. This study aims to report the experience of undergraduate and graduate students in dentistry at the Federal University of Paraná in the re-opening of the dental outpatient clinic for patients with hereditary coagulopathies and thrombopathies in the State of Paraná. With the retirement of the dentist accounting for local dental care, the patients were left unattended. To meet the repressed demand, the dental outpatient clinic was re-opened as an extramural activity for undergraduate and postgraduate courses in dentistry. This fact made it possible to improve the students' care for patients with systemic impairment, in addition to resuming dental care for patients with predisposition to haemorrhage. The re-opening of a highly complex clinic as a field of academic practice is atypical, as the process commonly takes place in reverse order, that is, with the student entering well-established service. In the present experience, the students played a fundamental role in the re-establishment of dental care at the blood centre (AU).

Antimicrobial Photodynamic Therapy as a treatment option for inoperable cases of medication-related Osteonecrosis of the jaws.

The present study aims to report conservative treatment of an extreme case of MRONJ in a patient illegible for surgical treatment. A 69-year-old female patient, diagnosed with Multiple Myeloma underwent treatment with bisphosphonate. After a turbinectomy, she was referred to a dental clinic with painful oral symptoms, presence of necrotic and suppurative bone exposures in maxilla compatible with MRONJ. As surgery was not indicated, the treatment of option was to control infection and pain. PENTO protocol, antibiotic therapy and photodynamic therapy were instituted. The patient has been under dental follow-up for more than 1 year, with an important reduction in the areas of bone exposure, absence of active infection and painful symptoms, characterizing a significant improvement in the oral condition. The conservative approach achieved satisfactory results for the case, where the patient is ineligible for a surgery due to local and general conditions, and quality of life being prioritized.

Graft-versus-host disease and herpes simplex virus oral injuries in onco-hematological patient: Case report.

INTRODUCTION: Graft-versus-host disease (GVHD) is a systemic complication that can affect patients undergoing allogeneic hematopoietic stem cell transplantation (HSCT) and the mouth is one of the places affected by the disease (oGVHD). Topical corticosteroids are used to control the painful symptoms, causing a local immunosuppression and increasing the risk for opportunistic infections. OBJECTIVE: This study aims to report a case of a 42-year-old woman, diagnosed with Chronic Myeloid Leukemia, who developed oGVHD and herpes simplex virus (HSV) infection after HSCT. CASE REPORT: oGVHD was confirmed by incisional biopsy and viral infection by HSV types 1 and 2 was confirmed by PCR. Topical and systemic corticotherapy and antiviral therapies were instituted. The immunosuppression intensified viral infection and antimicrobial photodynamic therapy (aPDT) was instituted as an adjuvant treatment. aPDT application, along with the patient's immune recovery, antivirals and corticosteroids resulted in clinical improvement of oral lesions. FINAL CONSIDERATIONS: This study highlights aPDT as an effective adjunct therapy in the local treatment of opportunistic infections in onco-hematological patients.

Lingual amyloidosis associated to multiple myeloma: case report / Amiloidose lingual associada a mieloma múltiplo: relato de caso

ABSTRACT Oral amyloidosis is a disease characterized by extracellular and irreversible deposition of amorphous and fibrillar proteins in the oral cavity, being strongly associated with Multiple Myeloma. The objective of this study is to report a case of a 62-year-old woman diagnosed with Multiple Myeloma who, approximately 2 years after starting treatment for the underlying disease, presented a lesion on the lateral border of the tongue with exophytic growth, pinkish color, vascularized, painless, measuring 3cm in its largest diameter. After histopathological analysis through incisional biopsy, a final diagnosis of amyloidosis was obtained. As a local treatment, we opted for complete excision of the lesion. The patient evolved to death due to Multiple Myeloma influenced by the diagnosis of systemic amyloidosis. Oral amyloidosis is usually associated with the systemic presentation of the disease, making it necessary to conduct a thorough investigation of other organs. Its diagnosis is important since the prognosis is directly related and can negatively influence survival rates and treatment of the underlying disease.

RESUMO A amiloidose oral é uma doença caracterizada pela deposição extracelular e irreversível de proteínas amorfas e fibrilares na cavidade bucal, sendo fortemente associada com Mieloma Múltiplo. O objetivo deste trabalho é relatar o caso de uma mulher com 62 anos de idade diagnosticada com Mieloma Múltiplo que, aproximadamente 2 anos após o início do tratamento para a doença de base, apresentou lesão em borda lateral de língua com crescimento exofítico, de coloração rósea, vascularizada, indolor, medindo 3 cm em seu maior diâmetro. Após análise histopatológica através de biópsia incisional, obteve-se diagnóstico final de amiloidose. Como tratamento local, optou-se pela exérese completa da lesão. A paciente evoluiu para óbito por consequência do Mieloma Múltiplo com complicações associadas à amiloidose sistêmica. A amiloidose oral geralmente está associada com a apresentação sistêmica da doença, tornando necessária uma investigação aprofundada nos demais órgãos. Seu diagnóstico é importante uma vez que pode influenciar diretamente no prognóstico e, consequentemente, negativamente nas taxas de sobrevida e no tratamento da doença de base.

A case series of medication-related fibrovascular hyperplasia following hematopoietic stem cell transplantation for Fanconi anemia.

Systemic medications categorized as diphenylhydantoin, calcineurin inhibitor and calcium channel blocker may have effects on the oral cavity by modifying the inflammatory and immune response and causing undesired tissue proliferative reactions. Calcineurin inhibitors are medications commonly used for long periods in patients undergoing allogeneic hematopoietic stem cell transplant (HSCT) and solid organ transplantation. Medication-related fibrovascular hyperplasia (MRFH) is an extra gingival hyperplastic nodular growth associated with medications use. This study reports five cases of pediatric patients (6 to 12-years-old) diagnosed with Fanconi anemia (FA) after HSCT who presented similar oral mucosal lesions associated with the use of cyclosporine, phenobarbital and amlodipine. After excision of the lesions, histopathological analysis described them as pyogenic granuloma (PG). As the aetiology of the lesions manifested by the patients was associated with the use of medications, the final diagnosis was MRFH. Despite the clinical and histopathological similarity between PG and MRFH, it is fundamental to know the aetiological agent for achieving definitive diagnosis and correct management. Considering the etiologic agent (medication) and histopathological findings, it is suggested that the most appropriate term for this manifestation should be "medication-related fibrovascular hyperplasia". The correct nomenclature related to extra gingival hyperplastic lesions identified in patients on medications with potential to induce hyperplastic reactions should be adopted to facilitate scientific communication and improve the treatment.

Periodontal Attachment Loss in Children with Amegakaryocytic Purpura: Case Reports of Two Male Siblings.

AIMS: To report the periodontal condition of two siblings (ages 2 and 4) diagnosed with congenital Amegakaryocytic Purpura (AP), who underwent allogeneic hematopoietic stem cell transplant (HSCT) and developed graft-versus-host disease (GVHD) with oral manifestations. METHODS: Clinical history was obtained through physical examination and medical records. Patients received clinical and microbiological assessment at 2 months post-HSCT, when they started to show signs and symptoms of GVHD and were monitored at 8/15-months post-transplant. They were treated by means of prophylaxis and oral hygiene instruction. Two supragingival biofilm samples were collected from each patient and analyzed by Checkerboard DNA-DNA hybridization. RESULTS: Patients developed severe periodontal clinical attachment loss (CAL) in deciduous dentition associated with recession of the periodontal tissues. They also presented GVHD lesions in the oral mucosa, lips and tongue. Caries lesions, gingivitis, and heavy biofilm deposits were identified. The microbiological profile of biofilm samples presented high levels and proportions of periodontal pathogens, such as Aggregatibacter actinomycetemcomitans. CONCLUSION: The cases presented suggested that severe periodontal CAL in children with AP may be an atypical manifestation associated with AP and/or GVHD, which may be aggravated by the presence of a dysbiotic biofilm containing periodontal pathogens, especially A. actinomycetemcomitans.

Medication-related fibrovascular hyperplasia lesion in tongue: Case report.

OBJECTIVE: The objective of this study is to report the case of a patient who underwent hematopoietic stem cell transplantation for Hodgkin's lymphoma treatment and developed multiple tongue lesions during recovery. METHODS AND RESULTS: This is the case report of a patient who developed ulcerated lesions with areas of depapillation on the border and dorsum of the tongue. The ulcer evolved to a reddish fibrous hyperplastic nodule, similar to adjacent mucosa. The patient was using a series of medications, such as antifungals, antibiotics, antivirals, corticosteroids, and analgesics in addition to immunosuppression with cyclosporine. Considering the medical history of the patient, a biopsy was performed. Histopathological analyses describe hyperplasia, granulation tissue, vascular proliferation, and intense inflammatory infiltrate, and the diagnosis was of medication-related fibrovascular hyperplasia (MRFH). CONCLUSION: Patients in use of cyclosporine are at risk to develop oral lesions, such as MRFH. The correct diagnosis is important, so the adequate treatment and follow-up are instituted even considering the immunosuppression protocol.