ABSTRACT
IgG4-related disease is an inflammatory condition characterized by high levels of IgG4. It affects salivary and lacrimal glands, pancreas, lymph nodes, lungs or kidney. The diagnosis is based on identifying a histological pattern with a dense lymphocyte and plasmacyte infiltration, focal fibrosis or phlebitis, finding more than 10 IgG4 positive cells per high power field and/or IgG4/IgG ratio in plasma higher than 40%. We present a patient with Mikulicz's disease who meets histological findings required for the diagnosis of IgG4 related disease.
La enfermedad relacionada con IgG4 es una condición fibroinflamatoria en la que existe elevación de IgG4, afección a nivel de glándulas salivares, lacrimales, páncreas, ganglios linfáticos y pulmón. Para su diagnóstico se requiere la identificación de un patrón histológico sugestivo que muestre infiltrado linfoplasmocitario denso, fibrosis focal o flebitis a nivel de una glándula, más de 10 células positivas para IgG4 por campo de gran aumento y relación de IgG4/IgG arriba de 40% en plasma. Describimos el caso de una paciente que presentó enfermedad de Mikulicz y cumplió con los datos histológicos para diagnóstico de enfermedad relacionada con IgG4.
Subject(s)
Autoimmune Diseases/diagnosis , Immunoglobulin G/metabolism , Mikulicz' Disease/immunology , Autoimmune Diseases/complications , Autoimmune Diseases/metabolism , Biomarkers/metabolism , Female , Humans , Middle AgedABSTRACT
BACKGROUND: Epstein-Barr virus (EBV) is a member of the Herpesviridae family and is associated with Hodgkin lymphoma (HL). Isolates of EBV are classified according to sequence variation in the latency genes such as Epstein-Barr virus nuclear antigen (EBNA). EBNA2 contains the most divergent locus and is classified into type 1 and type 2 or EBNA2A and EBNA2B, respectively. We compared the frequency of EBV and the distribution of EBNA genotypes in Mexican children and adults with HL. PATIENTS AND METHODS: Lymph node biopsy specimens from children and adults with HL were embedded in paraffin. EBV was identified by LMP1 amplification and Epstein-Barr-encoded RNA EBER by in situ hybridization (ISH) and genotyped as EBNA2A or EBNA2B using nested polymerase chain reaction (PCR) and specific primers for the detection of subtype. RESULTS: Sixty-six samples were obtained from 3 hospitals-42 (63%) from children and 24 (37%) from adults with HL. Thirty-two of the 42 samples (76.1%) were positive for EBV in children and 16 of 24 (66.6%) samples were positive in adults (P = .41). In both children and adults, EBV was found more frequently in male patients. Thirty-four of 48 cases could be typed (70.8%). EBNA2A was found in 7/21 (33.3%) children and in 4/13 (30.8%) adults (P = 1.0), and EBNA2B was found in 10/21 (47.6%) children and in 9/13 (69.2%) adults (P = .22). A mix of subtypes was found in 4/21 (19%) children. CONCLUSION: EBV was found frequently in both children and adults with HL. EBNA2B was the most frequent subtype, and a high frequency of mixed subtypes was found in children.
Subject(s)
Epstein-Barr Virus Infections/virology , Epstein-Barr Virus Nuclear Antigens/genetics , Herpesvirus 4, Human/isolation & purification , Hodgkin Disease/virology , Viral Proteins/genetics , Adolescent , Adult , Child , Child, Preschool , Epstein-Barr Virus Infections/pathology , Female , Genotype , Herpesvirus 4, Human/genetics , Herpesvirus 4, Human/immunology , Hodgkin Disease/pathology , Humans , Immunohistochemistry , In Situ Hybridization , Male , Retrospective Studies , Young AdultABSTRACT
Non-Hodgkin lymphoma comprises a heterogeneous group of haematological malignancies, classified according to their clinic, anatomic-pathological features and, lately, to their molecular biomarkers. Despite the therapeutic advances, nearly half of the patients will die because of this disease. The new diagnostic tools have been the cornerstone to design recent therapy targets, which must be included in the current treatment guidelines of this sort of neoplasms by means of clinical trials and evidence-based medicine. In the face of poor diagnoses devices in most of the Mexican hospitals, we recommend the present diagnose stratification, and treatment guidelines for non-Hodgkin lymphoma, based on evidence. They include the latest and most innovative therapeutic approaches, as well as specific recommendations for hospitals with limited framework and therapy resources.
