Asunto(s)
Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Enfermedad Inmunoproliferativa del Intestino Delgado/patología , Linfoma/patología , Transformación Celular Neoplásica , Microbioma Gastrointestinal , Humanos , Enfermedad Inmunoproliferativa del Intestino Delgado/epidemiología , Linfoma/microbiología , Factores SocioeconómicosAsunto(s)
Inmunoglobulina A , Enfermedad Inmunoproliferativa del Intestino Delgado/patología , Linfoma Inmunoblástico de Células Grandes/patología , Linfoma Plasmablástico/patología , Anciano de 80 o más Años , Linfocitos B/inmunología , Linfocitos B/patología , Femenino , Humanos , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Enfermedad Inmunoproliferativa del Intestino Delgado/terapia , Neoplasias Intestinales/diagnóstico , Neoplasias Intestinales/inmunología , Neoplasias Intestinales/patología , Linfoma Inmunoblástico de Células Grandes/inmunología , Linfoma Inmunoblástico de Células Grandes/terapia , Linfoma Plasmablástico/inmunología , Linfoma Plasmablástico/terapiaRESUMEN
AIM: To evaluate roles of syndecan-1, bcl6 and p53 in diagnosis and prognostication of immunoproliferative small intestinal disease (IPSID) and to study profiles of kappa (kappa) and lambda (lambda) light chains and IgA heavy chain. METHODS: The study consisted of 11 cases of IPSID and similar number of controls which included 11 of normal intestinal mucosa and 11 of high grade B cell lymphoma of ileum. The parameters analyzed included clinical profiles, biochemical and other laboratory investigations, radiologic and histological findings including immunohistochemistry. RESULTS: All IPSID cases had demonstrable serum IgA heavy chain and heavy mucosal plasma cell infiltration. According to Galian's histological staging, there were 4 patients with stage A and 7 with stage B. kappa and lambda light chains were over-expressed in 7 patients; 1 stage A patient had H pylori-positive active gastritis and eradication of H pylori led to disease remission. Stage A biopsies had higher expression for syndecan-1, while stage B had higher expression for bcl6 and p53. Syndecan-1, kappa and lambda light chains and IgA heavy chain showed inverse relationship with bcl6 and p53. All patients were treated with doxycycline. CHOP regime was added in 5 patients who developed frank lymphoma. Three died of the disease due to extensive organ infiltration. CONCLUSION: Certain immunomarkers like syndecan-1, kappa and lambda light chains and IgA heavy chain could be of much help in identifying early stage IPSID. Stage B IPSID showed higher expression for bcl6 and p53 than stage A IPSID. bcl6 and p53 expressions correlated with a more advanced disease stage and aggressive tumour behavior.
Asunto(s)
Proteínas de Unión al ADN/genética , Enfermedad Inmunoproliferativa del Intestino Delgado/diagnóstico , Enfermedad Inmunoproliferativa del Intestino Delgado/genética , Glicoproteínas de Membrana/genética , Proteoglicanos/genética , Proteína p53 Supresora de Tumor/genética , Adulto , Antibacterianos/uso terapéutico , Estudios de Casos y Controles , Proteínas de Unión al ADN/análisis , Proteínas de Unión al ADN/inmunología , Progresión de la Enfermedad , Doxiciclina/uso terapéutico , Endoscopía Gastrointestinal , Femenino , Regulación de la Expresión Génica , Helicobacter pylori , Humanos , Cadenas alfa de Inmunoglobulina/sangre , Cadenas alfa de Inmunoglobulina/genética , Cadenas kappa de Inmunoglobulina/análisis , Cadenas kappa de Inmunoglobulina/genética , Cadenas lambda de Inmunoglobulina/análisis , Cadenas lambda de Inmunoglobulina/genética , Inmunohistoquímica , Enfermedad Inmunoproliferativa del Intestino Delgado/tratamiento farmacológico , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Mucosa Intestinal/química , Mucosa Intestinal/microbiología , Mucosa Intestinal/patología , Intestino Delgado/química , Intestino Delgado/microbiología , Intestino Delgado/patología , Masculino , Glicoproteínas de Membrana/análisis , Glicoproteínas de Membrana/inmunología , Persona de Mediana Edad , Pronóstico , Proteoglicanos/análisis , Proteoglicanos/inmunología , Proteínas Proto-Oncogénicas c-bcl-6 , Sindecano-1 , Sindecanos , Proteína p53 Supresora de Tumor/análisis , Proteína p53 Supresora de Tumor/inmunologíaRESUMEN
We report a case of the nonsecretory variant of immunoproliferative small intestinal disease involving the distal small bowel and the mesenteric and retroperitoneal lymph nodes in a 19-year-old woman from Mexico. This variant extranodal marginal zone B-cell lymphoma appeared similar in the different sites of involvement, with more interspersed large cells and greater plasmacytic differentiation present in intestinal specimens. Characteristic lymphoepithelial lesions and follicular colonization were seen in intestinal and lymph node sections, respectively. The neoplastic B cells were cytoplasmic immunoglobulin (Ig) A heavy-chain restricted and lacked surface and cytoplasmic light-chain expression by flow cytometric analysis. Serum and urine protein electrophoresis/immunofixation revealed hypogammaglobulinemia with no paraprotein. Molecular studies showed absence of immunoglobulin heavy-chain (IgH) gene rearrangement, with a nonfunctional clonotypic rearrangement of the kappa light-chain gene. This case highlights the role for kappa light-chain gene evaluation in immunoproliferative small intestinal disease, because IgH gene rearrangement analysis is often negative.
Asunto(s)
Enfermedad Inmunoproliferativa del Intestino Delgado/patología , Ganglios Linfáticos/patología , Linfoma de Células B de la Zona Marginal/patología , 2-Piridinilmetilsulfinilbencimidazoles , Adulto , Amoxicilina/uso terapéutico , Antibacterianos/uso terapéutico , Bencimidazoles/uso terapéutico , Quimioterapia Combinada , Femenino , Reordenamiento Génico de Cadena Ligera de Linfocito B/genética , Humanos , Inmunofenotipificación , Enfermedad Inmunoproliferativa del Intestino Delgado/genética , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Intestino Delgado/patología , Mesenterio , Metronidazol/uso terapéutico , Omeprazol/análogos & derivados , Omeprazol/uso terapéutico , Pantoprazol , Espacio Retroperitoneal , Sulfóxidos/uso terapéuticoRESUMEN
Immunoproliferative small intestinal disease (IPSID) was recently added to the growing list of infectious pathogen-associated human lymphomas. Molecular and immunohistochemical studies demonstrated an association with Campylobacter jejuni. IPSID is a variant of the B-cell lymphoma of mucosa-associated lymphoid tissue (MALT), which involves mainly the proximal small intestine resulting in malabsorption, diarrhea, and abdominal pain. Geographically, IPSID is most prevalent in the Middle East and Africa. IPSID lymphomas reveal excessive plasma cell differentiation and produce truncated alpha heavy chain proteins lacking the light chains as well as the first constant domain. The corresponding mRNA lacks the variable heavy chain (V(H)) and the constant heavy chain 1 (C(H)1) sequences and contains deletions as well as insertions of unknown origin. The encoding gene sequence reveals a deletion of V region and parts of C(H)1 domain. Cytogenetic studies demonstrated clonal rearrangements involving predominantly the heavy and light chain genes, including t(9;14) translocation involving the PAX5 gene. Early-stage IPSID responds to antibiotics (30%-70% complete remission). Most untreated IPSID patients progress to lymphoplasmacytic and immunoblastic lymphoma invading the intestinal wall and mesenteric lymph nodes, and may metastasize to a distant organ. IPSID lymphoma shares clinical, morphologic, and molecular features with MALT lymphoma, lymphoplasmacytic lymphoma, and plasma cell neoplasms.
Asunto(s)
Infecciones por Campylobacter , Campylobacter jejuni , Enfermedad Inmunoproliferativa del Intestino Delgado , Linfoma de Células B de la Zona Marginal , Células Plasmáticas/inmunología , Adolescente , Adulto , África , Infecciones por Campylobacter/complicaciones , Infecciones por Campylobacter/genética , Infecciones por Campylobacter/inmunología , Infecciones por Campylobacter/patología , Infecciones por Campylobacter/terapia , Niño , Cromosomas Humanos Par 14 , Cromosomas Humanos Par 9/genética , Cromosomas Humanos Par 9/inmunología , Femenino , Humanos , Cadenas Ligeras de Inmunoglobulina/genética , Cadenas Ligeras de Inmunoglobulina/inmunología , Región Variable de Inmunoglobulina/genética , Región Variable de Inmunoglobulina/inmunología , Cadenas alfa de Inmunoglobulina/genética , Cadenas alfa de Inmunoglobulina/inmunología , Enfermedad Inmunoproliferativa del Intestino Delgado/etiología , Enfermedad Inmunoproliferativa del Intestino Delgado/genética , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Enfermedad Inmunoproliferativa del Intestino Delgado/patología , Enfermedad Inmunoproliferativa del Intestino Delgado/terapia , Intestino Delgado/inmunología , Intestino Delgado/patología , Ganglios Linfáticos/inmunología , Ganglios Linfáticos/patología , Linfoma de Células B de la Zona Marginal/etiología , Linfoma de Células B de la Zona Marginal/inmunología , Linfoma de Células B de la Zona Marginal/patología , Linfoma de Células B de la Zona Marginal/terapia , Masculino , Mesenterio/inmunología , Mesenterio/patología , Medio Oriente , Factor de Transcripción PAX5/genética , Factor de Transcripción PAX5/inmunología , Células Plasmáticas/patología , Eliminación de Secuencia/genética , Eliminación de Secuencia/inmunología , Translocación Genética/genética , Translocación Genética/inmunologíaAsunto(s)
Infecciones por Campylobacter/complicaciones , Campylobacter jejuni , Infecciones por Helicobacter/complicaciones , Helicobacter pylori , Enfermedad Inmunoproliferativa del Intestino Delgado/microbiología , Linfoma de Células B de la Zona Marginal/microbiología , Linfocitos B/fisiología , Humanos , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Linfoma/microbiología , Linfocitos T/inmunologíaAsunto(s)
Cadenas alfa de Inmunoglobulina/análisis , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Mucosa Intestinal/inmunología , Linfocitos/patología , Linfoma de Células B/inmunología , Adulto , Progresión de la Enfermedad , Femenino , Humanos , Técnicas para Inmunoenzimas , Intestino DelgadoAsunto(s)
Antibacterianos/uso terapéutico , Enfermedad Inmunoproliferativa del Intestino Delgado/tratamiento farmacológico , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Tetraciclina/uso terapéutico , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Ciclofosfamida/administración & dosificación , Doxorrubicina/administración & dosificación , Femenino , Humanos , Prednisona/administración & dosificación , Vincristina/administración & dosificaciónRESUMEN
Immunoproliferative small intestinal disease (IPSID), a proliferative disorder affecting the intestinal immune system, has only been reported sporadically in India. Fifteen patients with malabsorption syndrome who were diagnosed to have IPSID were included in this study. Mucosal biopsies from all patients, full thickness surgical biopsies from 10 and autopsy material from four patients were examined by light microscopy and immunohistochemistry. The patients were predominantly young (aged 16-36 years) and male (13 of 15). Diarrhoea, weight loss, vomiting and abdominal pain were the major symptoms. The upper small bowel was involved in all cases. Involvement of large bowel was detected antemortem in three patients, but was found in all autopsied patients. Involvement of the stomach was noted in one patient at autopsy. Mesenteric lymph nodes were involved in all patients who underwent laparotomy. The plasmacytic infiltrate was uniformly positive for alpha-heavy chain, and either negative for light chain production or showed monotypic light chain production. Some of the blasts were also positive for alpha-heavy chain. Three patients died before therapy could be commenced. One patient with stage A disease is alive and clinically free of disease at 7 years. Of the remainder, there have been four long-term survivors with chemotherapy. Immunoproliferative small intestinal disease occurs in southern India and has characteristics similar to that in other parts of the world. Early diagnosis may improve outcome in this disease.
Asunto(s)
Enfermedad Inmunoproliferativa del Intestino Delgado/patología , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Biopsia , Ciclofosfamida/uso terapéutico , Doxorrubicina/uso terapéutico , Femenino , Humanos , Inmunohistoquímica , Enfermedad Inmunoproliferativa del Intestino Delgado/tratamiento farmacológico , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Enfermedad Inmunoproliferativa del Intestino Delgado/mortalidad , India , Ganglios Linfáticos/patología , Masculino , Estadificación de Neoplasias , Prednisona/uso terapéutico , Sobrevivientes , Tetraciclina/uso terapéutico , Vincristina/uso terapéuticoRESUMEN
Immunoproliferative small intestinal disease (IPSID), most common in Mediterranean countries, is characterized by lymphomatous infiltration of the small intestine and is usually associated with the synthesis of anomalous immunoglobulin alpha heavy chains. Flow cytometric analysis of DNA content, S phase fraction, and quantitative analysis of the proliferation-associated nuclear antigen, P105, were performed in 23 patients with IPSID to determine if they could be used as prognostic indicators in this disease. Eighteen patients had low-grade, two had intermediate-grade, and three had high-grade lymphoma. Eight patients had clinical stage IE disease, 12 had stage IIE, and three had stage IIIE disease. Eleven patients survived > 5 yr (good prognosis), four survived between 2-5 yr (intermediate prognosis), and eight survived 2 yr or less (poor prognosis). The S phase fraction of patients with poor prognosis was significantly higher than those with intermediate or good prognosis (P < 0.004). Flow cytometric evaluation of S phase fraction may offer important prognostic information in patients with IPSID and could be useful in the clinical management of patients with this highly variable clinical syndrome. Further studies evaluating the value of DNA flow cytometry in larger groups of patients with IPSID are warranted.
Asunto(s)
Antígenos de Neoplasias/análisis , ADN de Neoplasias/análisis , Enfermedad Inmunoproliferativa del Intestino Delgado/genética , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Antígeno Nuclear de Célula en Proliferación/análisis , Adulto , Femenino , Citometría de Flujo , Humanos , Enfermedad Inmunoproliferativa del Intestino Delgado/patología , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Valor Predictivo de las Pruebas , Pronóstico , Fase S , Coloración y Etiquetado , Análisis de SupervivenciaRESUMEN
Worsening of long-lasting diarrhea, abdominal discomfort and weight loss were main symptoms in a 27-year-old Moroccan woman who had lived in Germany for 18 years. Pseudomonas, salmonella and lamblia cysts were found in stools. Histological examination of the gastrointestinal tract showed immunoproliferative small intestinal disease (IPSID), characterized by atrophy of the villi and lymphoplasmocytic infiltrates. alpha 1-heavy chains were found immunohistologically in the biopsy specimen, but not in serum, urine or jejunal juice. HLA-typing gave evidence of A9. Antibiotic treatment was successful for almost one year. Clinical, histological and immunological diagnosis of IPSID in an African woman living for nearly 20 years in Europe shows that, besides environmental factors, genetic disposition is an essential factor in the development of IPSID.
Asunto(s)
Enfermedad Inmunoproliferativa del Intestino Delgado/patología , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Diagnóstico Diferencial , Femenino , Gastroenteritis/diagnóstico , Antígenos HLA-A/aislamiento & purificación , Humanos , Enfermedad Inmunoproliferativa del Intestino Delgado/tratamiento farmacológico , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Metronidazol/uso terapéuticoAsunto(s)
Enfermedades de las Vías Biliares/inmunología , Enfermedades Gastrointestinales/inmunología , Hepatitis/inmunología , Enfermedades Autoinmunes/inmunología , Humanos , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Enfermedades Inflamatorias del Intestino/inmunología , Mucosa Intestinal/inmunologíaRESUMEN
The effects of 3 experimental diets that varied only in the source of dietary protein (ie, poultry, cereal, red meat) were compared in Basenjis (n = 8) with immunoproliferative enteropathy and healthy Beagles (n = 8). Significant differences in fecal character, serum IgA concentration, and intestinal digestive and absorptive function were not induced by the different sources of dietary protein. The results of this study do not support a causal role for dietary protein source in the pathogenesis of immunoproliferative enteropathy of Basenjis.
Asunto(s)
Alimentación Animal , Proteínas en la Dieta/administración & dosificación , Enfermedades de los Perros/etiología , Enfermedad Inmunoproliferativa del Intestino Delgado/veterinaria , Animales , Peso Corporal , Enfermedades de los Perros/inmunología , Perros , Grano Comestible , Femenino , Enfermedad Inmunoproliferativa del Intestino Delgado/etiología , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Masculino , Carne , Aves de Corral , Distribución AleatoriaRESUMEN
A 69-year-old man with a rare case of lymphoma-type alpha-chain disease was admitted to the hospital with marked cervical and inguinal lymph node swelling. Lymph node biopsy showed marked infiltration of plasma cells, plasmacytoid cells and immunoblastoid cells, alone or in combination. Immunoelectrophoresis and immunoselection identified alpha-chain in the serum and urine. The site of alpha-chain synthesis was extensively studied in the whole body, and the immunoperoxidase technique eventually demonstrated the presence of alpha-chains in the cytoplasm of infiltrating malignant cells in the lymph nodes. No infiltrating malignant cells were found in other organs or tissues, including those of the digestive and respiratory tracts.
Asunto(s)
Enfermedad Inmunoproliferativa del Intestino Delgado , Anciano , Citoplasma/inmunología , Retículo Endoplásmico/inmunología , Humanos , Inmunoelectroforesis , Técnicas para Inmunoenzimas , Cadenas alfa de Inmunoglobulina/análisis , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Enfermedad Inmunoproliferativa del Intestino Delgado/patología , Ganglios Linfáticos/inmunología , Ganglios Linfáticos/patología , Masculino , Microscopía Inmunoelectrónica , Células Plasmáticas/patologíaAsunto(s)
Enfermedad Inmunoproliferativa del Intestino Delgado/patología , Diagnóstico Diferencial , Humanos , Enfermedad Inmunoproliferativa del Intestino Delgado/clasificación , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Enfermedad Inmunoproliferativa del Intestino Delgado/terapiaRESUMEN
We performed a detailed histological and immunohistological study on both fresh-frozen and paraffin-embedded tissue from full-thickness jejunal biopsy specimens taken from three patients with immunoproliferative small-intestinal disease (IPSID). In all three patients, the mucosal infiltrate consisted of "centrocyte-like" (CCL) cells forming lymphoepithelial lesions and plasma cells. In one patient, the mucosal infiltrate was strikingly follicular. Immunohistochemistry showed alpha 1 heavy chain, but no light chain, in the perinuclear space and cytoplasm of the CCL cells and in the plasma cells. In two patients, the plasma cells (but not the CCL cells) also contained alpha 2 heavy chain. In the case showing a follicular pattern, the extrafollicular CCL cells and most of the cells within the mucosal follicles expressed alpha 1 heavy chain, but a minor and variable population of cells expressed polytypic IgM. The dendritic reticulum cells stained for alpha 1 (but not alpha 2) heavy chain, mu chain, and both light chains. In all cases, the CCL cells did not stain for common acute lymphoblastic leukaemia antigen (CALLA); in the follicles, CALLA negative cells displaced a residual CALLA-positive population to the periphery and merged with the CALLA negative cells outside the follicles. These findings confirm the homology between IPSID and low-grade B-cell "Western" lymphomas arising in mucosa-associated lymphoid tissue; they suggest that the follicular pattern sometimes seen in these lymphomas is caused by selective colonization of reactive follicles by CCL tumor cells.
Asunto(s)
Enfermedad Inmunoproliferativa del Intestino Delgado/patología , Adolescente , Adulto , Anticuerpos Monoclonales , Femenino , Humanos , Cadenas Pesadas de Inmunoglobulina/análisis , Inmunohistoquímica , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Yeyuno/citología , Yeyuno/patología , Masculino , Células Plasmáticas/inmunologíaAsunto(s)
Enfermedad Inmunoproliferativa del Intestino Delgado/genética , Adulto , Linfocitos B/inmunología , Reordenamiento Génico , Humanos , Inmunoglobulina A/análisis , Cadenas alfa de Inmunoglobulina/genética , Cadenas kappa de Inmunoglobulina/genética , Enfermedad Inmunoproliferativa del Intestino Delgado/inmunología , Enfermedad Inmunoproliferativa del Intestino Delgado/patología , Intestino Delgado/inmunología , Intestino Delgado/patología , Ganglios Linfáticos/inmunología , Ganglios Linfáticos/patología , Masculino , ARN Mensajero/análisisRESUMEN
Immunoglobulin isotypes in serum and intestinal secretions of Basenji dogs with chronic diarrhea, asymptomatic Basenji dogs, and healthy control dogs were quantitated and their molecular sizes characterized to detect alpha-chain, gamma-chain, or mu-chain fragments. Quantitation of immunoglobulin isotypes in serum showed that affected Basenjis have significantly elevated serum IgA values as compared to asymptomatic Basenjis and normal control dogs. However, IgA concentrations in intestinal wash fluids were not significantly different for the three groups. Immunoelectrophoresis (IEP) and polyacrylamide gel electrophoresis (PAGE) demonstrated that virtually all IgA was in the dimeric form. Using IEP and immunoselection, we were unable to detect evidence for the presence of alpha-chain or other heavy-(H)-chain fragments. Hyperimmune serum obtained from rabbits immunized with serum or a globulin fraction of affected Basenjis also failed to detect H-chain fragments. The results of this study indicate that immunoproliferative enteropathy of Basenjis resembles closely the nonsecretory form of human immunoproliferative small intestinal disease (IPSID).