Demência rapidamente progressiva por linfoma primário do sistema nervoso central: um relato de caso / Rapidly progressive dementia due to primary central nervous system lymphoma: a case report

As demências rapidamente progressivas são condições que impactam a vida do paciente, entretanto nem sempre são consideradas durante a investigação da síndrome demencial. Este relato descreve uma paciente portadora de artrite reumatoide em uso crônico de corticoterapia que evoluiu com um quadro de demência rapidamente progressiva no período de cinco meses. Durante a investigação, evidenciou-se o linfoma primário de sistema nervoso central como causa de sua síndrome demencial. Faz-se importante destacar que o uso prolongado de corticoide pode estar associado a um estado de imunossupressão, o que é fator de risco para o surgimento do linfoma de sistema nervoso central.

Rapidly progressive dementias are conditions that worsening the patient's life; however they are not always considered during the investigation of dementia syndrome. This report describes the case of a patient with rheumatoid arthritis and chronic use of corticosteroid therapy, who developed rapidly progressive dementia within 5 months. During the investigation, the primary lymphoma of the central nervous system was evidenced as the cause of its dementia syndrome. Importantly, prolonged use of corticosteroids may be associated with a state of immunosuppression, which is a risk factor for the onset of central nervous system lymphoma.

Monoartritis del codo e infarto óseo como presentación inicial de enfermedad linfoproliferativa / Elbow monoarthritis and bone infarction as a form of presentation of B cell lymphoma: case report

We report a 68-year-old woman presenting with pain and swelling in her left elbow. An elbow magnetic resonance with gadolinium evidenced bone marrow infiltration and a bone infarct. Given these findings, a body CT scan was performed which showed multiple mesenteric adenopathies and a large retroperitoneal mass. A lymph node biopsy confirmed a B cell lymphoma. Monoarthritis with no systemic manifestations represents a highly uncommon form of presentation of lymphoma. Moreover it usually affects inferior limbs, particularly in the presence of bone infarction.

The coexistence of hypercalcemia and hypoglycemia in a patient with a renal tumor and B cell lymphoma

SUMMARY Paraneoplastic syndromes are a heterogeneous group of malignant diseases caused by events which involve endocrine, immune and metabolic aspects and whose symptoms vary according to the substance produced and the primary tumor. Hypercalcemia is a frequent complication in cancer patients. Prognosis of cancer patients with hypercalcemia is usually poor. A factor called parathyroid hormone related peptide, whose actions are similar to those of the parathyroid hormone, is thought to be the most common cause of malignancy associated hypercalcemia. Non-islet hypoglycemic cell tumor consists of a rare syndrome characterized by the presence of a solid tumor and severe fasting hypoglycemia determined by an insulin-independent pathway. We report a case of a 59-year-old-man with a renal tumor and a T-cell rich large B cell lymphoma who was hospitalized due to severe hypercalcemia and hypoglycemia. The laboratory examination reported hypercalcemia with inhibited PTH and hypoglycemia with inhibited insulin secretion, arriving to the conclusion of tumoral peptide production. He received denosumab and corticoid therapy. The patient died one month later despite initial improvement after medical treatment. While a single paraneoplastic manifestation may be expected in most tumors, the coexistence of two or more of them is rare, except in hepatocellular carcinomas, and it has not yet been described in renal tumors.

Linfomatosis intravascular cerebral como causa de demencia rápidamente progresiva: reporte de un caso / Cerebral intravascular lymphomatosis as cause of subacute dementia: report of a case

Cerebral Intravascular Lymphomatosis is a type of non Hodgkin Lymphoma, generally composed with B cells, its ocurrence is infrequent, clinically progressive and has a fatal course. Vascular damage is related with tumoral infiltration and small caliber occlusion, with results in multi infarcts. Neurological symptoms and signs are frequently the first clinical manifestation, which include sub acute encephalopathy, cognitive impairment, delirium, aphasia, hemipharesis, visual disturbances, paraplegia, paresthesia and cranial nerves involvement. MRI shows images of vasculitis from CNS. Cerebral Intravascular Lymphomatosis must be considered in differential diagnosis of rapid and subacute dementias, and clinical cases with small vessel recurrent multi infarct of unusual etiology. We present the clinical case of a patient with rapid progressive dementia and systemic disease manifestation. The CNS involvement was characterized as a subacute encephalopathy, with confusion and agitation, seizures, motor disturbances, bilateral plantar extensor reflexes and cerebellar signs. The clinical symptomatic course was progressive, with weight loss and fluctuant fever. The patient had a fatal course after he was treated with Methylprednisolone. Postmortem pathologic examination revealed a diffuse non Hodgkin lymphoma of B cells, intravascular variant, with brain compromise, cerebellum, suprarenal glands, pancreas, myocardium, thyroid gland, lung kidney and the lever...

La linfomatosis intravascular cerebral es un tipo de Linfoma no Hodgkin generalmente de células B, de presentación infrecuente, curso clínico progresivo y fatal. El daño vascular es producto de oclusión de vasos de pequeño calibre por infiltración tumoral, con resultado de múltiples infartos. Las manifestaciones neurológicas suelen ser la forma de presentación clínica inicial, caracterizadas por un amplio espectro que incluye encefalopatía subaguda, deterioro cognitivo, delirio, afasia, hemiparesia, trastornos visuales, paraplejia, parestesias y compromiso de pares craneanos. Los estudios de RNM evidencian alteraciones indistinguibles de las vasculitis del SNC. La linfomatosis intravascular debe considerarse en el diagnóstico diferencial de las demencias rápidamente progresivas y en los cuadros de multi infarto cerebral de pequeño vaso de etiología inusual y recurrente. Se presenta el caso de un paciente con demencia rápidamente progresiva, con manifestaciones neurológicas de deterioro cognitivo y enfermedad sistémica. El compromiso del SNC se expresó como encefalopatía subaguda con confusión, agitación, y crisis convulsivas, trastornos motores con reflejo cutáneo plantar extensor bilateral y compromiso cerebeloso, de curso clínico sintomático progresivo, con baja de peso y fiebre fluctuante. El paciente fue sometido a tratamiento con metilprednisolona a pesar del cual falleció. El estudio necrópsico demostró alteraciones por Linfoma no Hodgkin difuso de células B, variante intra vascular, con compromiso del cerebro, cerebelo, glándulas suprarrenales, páncreas, miocardio, tiroides, pulmón riñón e hígado...

Linfoma primário não Hodgkin em ventrículo esquerdo detectado ao ecocardiograma em pacientes submetido a transplante cardíaco / Primary non-Hodgkin lymphoma in the left ventricle detected by echocardiography in a patient after heart transplant

O caso ilustra o papel do ecocardiograma no diagnóstico e acompanhamento terapêutico de linfoma primário intracardíaco, em pacientesubmetido a transplante cardíaco há 11 anos. No ecocardiograma transtorácico foi visibilizada massa que ocupava a cavidade ventricular esquerda, confirmada por ecocardiograma transesofágico e tomografia computadorizada. Não havia disseminação do tumor em outrosórgãos. A massa foi ressecada cirurgicamente e enviada para análise histopatológica e imuno-histoquímica que revelou linfoma não Hodgkin. O ecocardiograma, após a cirurgia, comprovou ausência do tumor, com função ventricular esquerda preservada. O paciente veio a falecer, quatro meses após, por complicações infecciosas. Destacamos a raridade desta apresentação clínica do paciente transplantado, sobretudo a localização no ventrículo esquerdo e o diagnóstico em vida possibilitado pelo ecocardiograma.

Primary cardiac diffuse large B-cell lymphoma with activated B-cell-like phenotype.

Primary cardiac lymphoma (PCL) is a rare and fatal disorder. It may often mimic other common cardiac tumors like cardiac myxoma because of similarities in the clinical presentation. We report a case of PCL of diffuse large B-cell type, in a 38-year-old, immunocompetent male who presented with superior vena cava syndrome that was excised as a myxoma. Histology revealed a large cell population diffusely and strongly expressing CD45, CD20, MUM1/IRF4 and FOXP1 hinting at an activated B-cell (ABC)-like phenotype. After four cycles of Rituximab with CHOP (cyclophosphamide, hydroxydaunorubicin, Oncovin, and prednisolone) the tumor regressed completely but the patient had a relapse and subsequently succumbed to the disease confirming the aggressive nature. The aggressive behavior of PCL may be possibly linked to its ABC-like origin.

Síncope y bocio nodular: desafío diagnóstico / Syncope associated to a thyroid nodule: report of one case

We report a 60 years old male presenting with a history of repeating syncopal episodes lasting seven days. The patient had a history of an intestinal lymphoma treated four years ago. Imaging studies showed a macro nodule in the left thyroid lobe. The pathological study disclosed a type B non Hodgkin lymphoma. The patient was treated with chemotherapy with a favorable outcome. The association of syncope and cervical tumors has been described previously.

Linfoma primario de tiroides: reporte de cuatro casos / Primary thyroid lymphoma: report of four cases

Primary thyroid lymphoma is uncommon and usually of non-Hodgkin type. We report four female patients with thyroid lymphoma. An 81 year-old patient that was operated and received three cycles of chemotherapy and was lost from follow up. A 16 year-old patient that is operated and received full chemotherapy and was discharged free of disease. A 80 year-old patient that was operated and died due to a multiple organ failure, 50 days after hospital admission and a 54 year-old female that was operated but rejected chemotherapy and was lost from follow up.

Combined central retinal artery and vein occlusion secondary to systemic non-Hodgkin's lymphoma.

We report a rare case of low-grade systemic B-cell non-Hodgkin's lymphoma (NHL) causing central retinal artery and vein occlusion, which was the only manifestation of disease recurrence. A young man with resolved systemic NHL underwent fluorescein angiography, magnetic resonance imaging and computed tomography to investigate a severe unilateral visual loss. A combined vascular occlusion was observed in the right eye. Neuroimaging detected optic nerve infiltration; but no systemic/ central nervous system involvement was observed. The patient was treated with high-doses of corticosteroids and optic nerve irradiation. The optic neuropathy and vascular occlusion were resistant to treatment. The subsequent neovascular glaucoma was treated by panretinal photocoagulation, which relieved the pain, but vision was not recovered. No further recurrence was observed over the following year.

Diffuse large B-cell lymphoma arising from a multicentric mixed variant of Castleman's disease.

This case report describes a patient with multicentric mixed type Castleman's disease and concomitant non-Hodgkin's lymphoma of diffuse large B cell type in the neck. Multicentric CD is a systemic illness with disseminated lymphadenopathy; its aggressive and usually fatal course is associated with infectious complications and risk for malignant tumors, such as lymphoma or Kaposi sarcoma.

Linfomas No-Hodgkin de células B y T primarias intestinales e infección por virus de Epstein Barr en la población uruguaya / B-cell and Primary T-cell intestinal no-Hodgkin lymphomas and Epstein Barr virus infection in the Uruguayan population

El propósito del presente trabajo es establecer si existe asociación entre los linfomas No-Hodgkin (LNH), a células B y T, y la infección por el virus de Epstein Barr (VEB) en la población uruguaya. El VEB se encuentra universalmente asociado con linfoma tipo Burkitt, periférico de células T, carcinoma nasofaríngeo indiferenciado y linfomas "Natural Killer", mostrando importante variación geográfica. La Enfermedad de Hodgkin (EH), y los linfomas B esporádicos procedentes de América Latina, han evidenciado mayores tasas de asociación con la infección por VEB que los originados en los países occidentales. En los LNH de células T de origen gastrointestinal, la frecuencia en la positividad para el VEB está influida por el sitio anatómico del tumor primario y el fenotipo de la célula neoplásica, siendo independiente de la presencia o ausencia de enteropatia. Analizamos 14 casos de linfomas a células B y T primarios intestinales en pacientes HIV negativos, diagnosticados por microscopía óptica y e inmunomarcación, evaluando la coexistencia con el VEB con técnicas de inmunohistoquimica (IHQ) con EBV LMP-1. En dos (14,3 por ciento) de los casos se comprobó esta asociación, siendo los restantes negativos para la infección por VEB. Los dos linfomas con evidencia de infección por el VEB fueron: uno, en mujeres y, el otro, en hombres, ambos de fenotipo a células B y ambos del subtipo difuso a grandes células, el subtipo que representó 72 por ciento de los linfomas estudiados.

Extranodal B-cell lymphoma in a patient with atopic-like dermatitis

Atopic dermatitis is a chronic relapsing skin disease that occurs commonly during early infancy and childhood and is frequently associated to personal and family history of allergic rhinitis, asthma and/or skin disease. As a rule, it follows a benign course. We report a case of a female patient with a chronic atopic-like dermatitis who eventually developed an extranodal systemic large B-cell lymphoma during the course of her disease

Diffuse large B cell lymphoma in a patient with hypocomplementemic urticarial vasculitis.

Hypocomplementemic urticarial vasculitis (HUV) is known to be associated with malignancies. Urticarial vasculitis has been linked to lymphomas, but to our knowledge, the association of HUV and non-Hodgkin lymphoma has not been described so far. A patient with HUV who developed 10 years later a diffuse large B cell lymphoma is reported here.

Mucinosis folicular generalizada / Generalized folicular mucinosis

Las lesiones de mucinosis folicular suelen localizarse en la cabeza o más raramente generalizarse a cuello, tronco y extremidades. Los hallazgos histológicos se caracterizan por la degeneración reticular del folículo pilosebáceo y el depósito de glicosaminoglicanos (mucina). Se presenta un caso de mucinosis folicular generalizada en un paciente varón de 21 años de edad. El examen reveló múltiples placas alopécicas bien definidas, eritematosas, formadas por pápulas queratósicas foliculares. Se destaca la gran extensión, el número de lesiones y la necesidad de seguimiento clínico e histológico ante la posibilidad de transformación en un linfoma cutáneo

Hepatosplenic B-cell lymphoma associated with hemophagocytic syndrome: a case report

While T-cell non-Hodgkin's lymphoma (NHL) associated with hemophagocytic syndrome (HPS) has been frequently observed, B-cell NHL associated with HPS has been rarely reported. We report a case of hepatosplenic B-cell lymphoma associated with HPS in a 41-year-old woman who presented with fever of unknown origin. An abdominal CT scan revealed splenomegaly with focal splenic infarction. Splenectomy and a liver wedge biopsy showed sinusoidal-pattern infiltration of medium to large tumor cells with positive reaction to a B-lymphocyte marker. Findings on bone marrow examination showed proliferation of histiocytes with avid hemophagocytosis.

Malignant lymphoma associated with hanseniasis

There are few reports in the world medical literature concerning association between leprosy and malignant lymphoma. It seams that, although defects in immune system related to leprosy are multiple and complex, no increase in incidence of malignant lymphoma among leprosy patients can be detected. This is also true for Brazil, where leprosy is an endemic disease, which poses an important public health problem. For this reason, the authors report on three cases of malignant lymphoma: one low grade, B-cell lymphoma (immunocytoma): one high grade, T-cell lymphoma and one mixed cellularity Hodgkin's disease in patients previously diagnosed as having leprosy.