RESUMO
BACKGROUND/AIMS/METHODS: Aggressive systemic mastocytosis (ASM) is a subtype of systemic mastocytosis, which comprises a heterogenous group of disorders characterized by infiltration of bone marrow, skin, liver, spleen, lymph nodes and gastrointestinal tract by neoplastic mast cells. There is lack of data on the association of ASM with renal involvement, as kidney is not among the known organs affected by ASM. RESULTS/CONCLUSIONS: To the best of our knowledge, this is the first case of ASM associated with mesangioproliferative glomerulonephritis and monoclonal gammopathy of undetermined significance, without the presence of nephrotic syndrome. The patient's clinical course and the intriguing family history, along with the treatment selection are described. Finally, the proposed possible pathophysiological mechanisms explaining the renal involvement of our patient are discussed.
Assuntos
Glomerulonefrite/patologia , Mastocitose Sistêmica/diagnóstico , Células Mesangiais/patologia , Idoso , Evolução Fatal , Feminino , Glomerulonefrite/terapia , Humanos , Mastocitose Sistêmica/terapia , Gamopatia Monoclonal de Significância IndeterminadaRESUMO
PURPOSE: To present a treatment plan for localized mucosa-associated lymphoid tissue lymphoma of the orbit in order to preserve vital structures and function from the side effects of radiotherapy. MATERIALS AND METHODS: Study of 2 clinical cases by means of clinical and radiologic examination, with 2 and 3 years follow-up, respectively. RESULTS: Immediate remission of the disease after 6 cycles of chemotherapy, with no signs of recurrence after 2 and 3 years. Clinical examination of the oculomotor mechanism and visual activity gave excellent results. CONCLUSION: Although radiotherapy is preferable for localized lymphoproliferative lesions, chemotherapy should also be considered as an effective treatment that preserves the integrity and function of the ocular adnexa.
Assuntos
Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Neoplasias Orbitárias/tratamento farmacológico , Idoso , Antígenos CD/análise , Biomarcadores Tumorais/análise , Quimioterapia Combinada , Exoftalmia/etiologia , Feminino , Humanos , Linfoma de Zona Marginal Tipo Células B/patologia , Linfoma de Zona Marginal Tipo Células B/cirurgia , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/prevenção & controle , Tomografia Computadorizada por Raios X , Resultado do TratamentoAssuntos
Aspergilose/diagnóstico por imagem , Leucemia Mieloide Aguda , Embolia Pulmonar/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Aspergilose/microbiologia , Aspergilose/patologia , Criança , Feminino , Humanos , Leucemia Mieloide Aguda/diagnóstico por imagem , Leucemia Mieloide Aguda/microbiologia , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/microbiologia , Artéria Pulmonar/patologia , Embolia Pulmonar/microbiologia , Embolia Pulmonar/patologia , Tomografia Computadorizada por Raios X/métodosAssuntos
Anticorpos Monoclonais/uso terapêutico , Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Aplasia Pura de Série Vermelha/complicações , Aplasia Pura de Série Vermelha/tratamento farmacológico , Anticorpos Monoclonais Murinos , Antígenos CD20/imunologia , Humanos , Masculino , Pessoa de Meia-Idade , Rituximab , Resultado do TratamentoRESUMO
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder characterized by pancytopenia, hemolysis, and thrombosis. Abdominal vein thrombosis is a life-threatening manifestation of this disease. We present a patient with complete spleen necrosis due to thrombosis of the splenic vessels. After splenectomy, other causes of thrombophilia were excluded and the diagnosis of PNH was established. The patient was put on anticoagulation but despite the prophylactic international normalized ratio maintained over the last 18 months of follow-up, he had another episode of intrahepatic thrombosis which was treated with tissue plasminogen activator thrombolysis.
