ABSTRACT
Los linfomas primarios del intestino delgado son tumores raros, ocupan del 20 al 40 por ciento de las neoplasias malignas de esa región. Son un grupo heterogéneo de tumores con características clínicas y patológicas variables. Son neoplasias de adultos, se presentan con mayor frecuencia de los 20 a los 60 años. Los síntomas se desarrollan insidiosamente y pueden incluir pérdida de peso, diarrea, sangrado rectal, dolor abdominal, vómito y constipación. La enfermedad celiaca, la enfermedad inflamatoria intestinal y los estados de inmunodeficiencia se consideran factores predisponentes. El comportamiento biológico, el cuadro clínico, la morfología, el inmunofenotipo y el tratamiento son diferentes de los de los linfomas originados en ganglios linfáticos. Para clasificarlos correctamente es necesario definir el tipo histológico, el grado histológico, la resecabilidad del tumor y si se logra o no la remisión después del tratamiento combinado
Subject(s)
Humans , Male , Female , Child, Preschool , Adolescent , Adult , Middle Aged , Immunoproliferative Small Intestinal Disease/pathology , Lymphoma, B-Cell/physiopathology , Lymphoma, B-Cell/pathology , Lymphoma, T-Cell/physiopathology , Lymphoma, T-Cell/pathology , Lymphoma, T-Cell/therapy , Burkitt Lymphoma/physiopathology , Burkitt Lymphoma/pathology , Burkitt Lymphoma/therapy , Survivors , Intestinal Neoplasms/diagnosis , Intestinal Neoplasms/physiopathology , Intestinal Neoplasms/pathology , Intestinal Neoplasms/therapy , Intestine, Small/pathology , Lymphoma/classification , Neoplasm Staging/adverse effects , PrognosisABSTRACT
Se presentan dos casos de linfoma primario del intestino delgado, evaluados y tratados en el hospital Vargas de Caracas, destacando los hallazgos clínicos y paraclínicos encontrados. Ambos casos mostraron una afección difusa del intestino delgado (IPSID), siendo clasificados histológicamente como linfoma no Hodgkin estadio IV, y tratados con quimioterapia. Se hace una revisión de esta patología
Subject(s)
Humans , Male , Female , Immunoproliferative Small Intestinal Disease/pathology , Immunoproliferative Small Intestinal Disease/therapy , Intestinal Neoplasms/pathology , Lymphoma , Lymphoma, Non-Hodgkin , GastroenterologyABSTRACT
O linfoma intestinal do Mediterrâneo é doença extremamente rara no Ocidente. Caracteriza-se pela existência de um infiltrado linfoplasmocitário difuso e intenso na mucosa do intestino delgado e/ou linfonodos mesentéricos. Difere do linfoma intestinal dito ocidental por ser endêmico na regiäo do Mediterrâneo e Oriente Médio, ocorrer em faixa etária mais jovem, acometer preferencialmente o intestino proximal e cursar com má absorçäo. Está relacionado com a doença das cadeias pesadas alfa. A oportunidade que tivemos de diagnosticar e acompanhar por cerca de um ano uma paciente de 19 anos com esta afecçäo motiva a presente publicaçäo. Ésta relacionado com a doença das cadeias pesadas alfa. A oportunidade que tivemos de diagnosticar e acompanhar por cerca de um ano uma paciente de 19 anos com esta afecçäo motiva a presente publicaçäo. É realizada extensa revisäo bibliográfica
Subject(s)
Humans , Adult , Female , Immunoproliferative Small Intestinal Disease/diagnosis , Diagnosis, Differential , Immunoproliferative Small Intestinal Disease/pathologyABSTRACT
Seventy-five cases of lymphoreticular lesions of the gastrointestinal tract were studied by the immunoperoxidase method on paraffin sections which demonstrated cytoplasmic markers, in particular immunoglobulin (Ig) in B-lymphocytes and alpha-1-antichymotrypsin in histiocytes. There were 4 cases of benign immunoproliferative disease (IPD), 1 in the stomach, 3 in the small intestine; their reactions were polyclonal. Large cell non-Hodgkin's lymphoma was subdivided into 21 cases with IPD ("Mediterranean Abdominal Lymphoma") and 29 cases without. Eight cases of lymphoma with IPD were gastric and 13 intestinal; 7 gastric and 12 intestinal lymphomas were of B-cell origin; 1 gastric tumour was histiocytic; 1 intestinal neoplasm had no detectable marker. Sixteen cases of lymphoma without IPD were gastric and 13 intestinal; 4 gastric and 4 intestinal lymphomas were of B-cell type; 3 gastric and 2 intestinal neoplasms were histiocytic; 1 intestinal tumour was a composite of B-cells and histiocytes, in separate but contiguous foci; 9 gastric and 4 intestinal lymphomas had no identifiable marker. One of 18 cases of mixed and small cell lymphoma was accompanied by IPD. In these types of lymphoma Ig was found only in 10-30% of cells, mainly with plasmacytoid differentiation.