RESUMO
This study was undertaken to summarize the current status of the clinicopathologic factors related to non-Hodgkin's lymphoma (NHL) development in patients with Sjögren's syndrome (SS). Through a MEDLINE search, pertinent articles on SS, monoclonality, and NHL were found and reviewed. Malignant lymphoma description was based on the International Lymphoma Study Group classification. Patients with SS are known to have an increased risk for developing B-cell NHL (B-NHL). However, such a complication occurs in less than 10% of patients, being reported mainly in those with primary SS. Extranodal low-grade B-NHL are observed most frequently. Persistent enlargement of parotid glands, adenopathy, monoclonal gammopathy, and cross-reactive idiotypes are all signs suggesting possible lymphoma evolution. Although monoclonality does not mean malignancy unequivocally, it is considered to be a precursor for NHL development in SS. Factors implicated into lymphomagenesis in SS include dysregulation in the mechanisms leading to apoptosis, hyperstimulation of B-1 cells, and an infectious agent. Polyclonal lymphoproliferation characterizing SS might in some instances transform into monoclonal, and then to malignancy. Further studies on the mechanism whereby NHL develops in SS are warranted.
Assuntos
Linfoma não Hodgkin/complicações , Síndrome de Sjogren/complicações , Idoso , Doenças Autoimunes/complicações , Doenças Autoimunes/imunologia , Humanos , Linfoma não Hodgkin/imunologia , Pessoa de Meia-Idade , Síndrome de Sjogren/imunologiaAssuntos
Doença de Hodgkin , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ensaios Clínicos como Assunto , Terapia Combinada , Feminino , Doença de Hodgkin/diagnóstico , Doença de Hodgkin/mortalidade , Doença de Hodgkin/patologia , Doença de Hodgkin/terapia , Humanos , Infertilidade/etiologia , Masculino , Estudos Multicêntricos como Assunto , Estadiamento de Neoplasias , Neoplasias Induzidas por Radiação/etiologia , Segunda Neoplasia Primária/etiologia , Radioterapia/efeitos adversos , Radioterapia/métodos , Células de Reed-Sternberg/patologia , Indução de Remissão , Terapia de Salvação , Taxa de SobrevidaAssuntos
Febre Maculosa das Montanhas Rochosas/diagnóstico , Aspartato Aminotransferases/sangue , Pré-Escolar , Ensaios Enzimáticos Clínicos , Creatina Quinase/sangue , Humanos , L-Lactato Desidrogenase/sangue , Fígado/enzimologia , Masculino , Febre Maculosa das Montanhas Rochosas/sangue , Manifestações Cutâneas , Fatores de TempoRESUMO
Thirty cases of malignant lymphoma, undifferentiated, Burkitt's type are reviewed. An older median age and a predominance of presentation in abdominal and pelvic sites rather than in the jaw distinguishes this series of American patients from those reported from endemic regions in Africa. Bone marrow involvement invariably consisted of massive infiltration recognizable in smear, clot and biopsy preparations. Involvement of the central nervous system or bone marrow was always associated with short survival. In all eight long-term survivors lymphoma was apparently confined to a single site at presentation. At autopsy, the most consistent finding was widespread multiorgan involvement without predilection for lymphoreticular structures. The histologic appearance of the tumor changed after chemotherapy, varying from diffuse necrosis within 48 hours of initial therapy to extreme pleomorphism of tumor cells after 9 months of therapy. In one patient, there was almost complete absence of lymphoma at autopsy in an organ site shown clinically to have been extensively involved by tumor prior to treatment. The diagnostic and therapeutic implications of these findings are discussed.
Assuntos
Linfoma de Burkitt/patologia , Abdome , Adolescente , Adulto , Autopsia , Biópsia , Medula Óssea/patologia , Criança , Pré-Escolar , Citoplasma/ultraestrutura , Feminino , Humanos , Corpos de Inclusão/ultraestrutura , Neoplasias Intestinais/patologia , Intestino Delgado/patologia , Fígado/patologia , Metástase Linfática , Macrófagos/patologia , Masculino , Neoplasias Mandibulares/patologia , Neoplasias Maxilares/patologia , América do Norte , Neoplasias Ovarianas/patologia , América do Sul , Estados UnidosRESUMO
The presenting clinical characteristics and the results of therapy in 30 cases of American Burkitt's lymphoma are described. Five patients presented with localized disease. The abdomen was the most frequent site of involvement (19 cases). Serum lactic dehydrogenase (LDH) levels closely correlated with extent of tumor mass. Of the 22 patients treated with large doses of parenteral cyclophosphamide, complete remission was achieved in 13 (59 per cent). Of these only four have had a relapse, all within 12 months of treatment. The remainder are alive, free of disease and have not received any treatment for up to 80 months or more. The site and volume of tumor mass predicted for prolonged survival. None of the six patients with bone marrow or central nervous system involvement remained tumor-free. A complete remission was achieved in 8 of 9 patients with presenting LDH levels of less than 700 IU/ml and they have remained free of disease, whereas only 4 of 13 patients with LDH levels greater than 700 IU/ml had a complete response and 3 of these had a relapse within 12 months. In six cases, the massive tumor regression following chemotherapy was associated with serious metabolid consequences including hyperkalemia (six cases), hypocalcemia, hyperphosphatemia (one case) and lactic acidosis (one case). There were four sudden deaths in less than 48 hours after chemotherapy; two of these were attributable to hyperkalemia. In all cases therw were large tumor masses and/or elevated serum LDH levels.