IgG4-Related Sclerosing Disease, an Emerging Entity: A Review of a Multi-System Disease

Immunoglobulin G4-related systemic disease (IgG4-RSD) is a recently defined emerging entity characterized by a diffuse or mass forming inflammatory reaction rich in IgG4-positive plasma cells associated with fibrosclerosis and obliterative phlebitis. IgG4-RSD usually affects middle aged and elderly patients, with a male predominance. It is associated with an elevated serum titer of IgG4, which acts as a marker for this recently characterized entity. The prototype is IgG4-related sclerosing pancreatitis or autoimmune pancreatitis (AIP). Other common sites of involvement are the hepatobiliary tract, salivary gland, orbit, and lymph node, however practically any organ can be involved, including upper aerodigestive tract, lung, aorta, mediastinum, retroperitoneum, soft tissue, skin, central nervous system, breast, kidney, and prostate. Fever or constitutional symptoms usually do not comprise part of the clinical picture. Laboratory findings detected include raised serum globulin, IgG and IgG4. An association with autoantibody detection (such as antinuclear antibodies and rheumatoid factor) is seen in some cases. Steroid therapy comprises the mainstay of treatment. Disease progression with involvement of multiple organ-sites may be encountered in a subset of cases and may follow a relapsing-remitting course. The principal histopathologic findings in several extranodal sites include lymphoplasmacytic infiltration, lymphoid follicle formation, sclerosis and obliterative phlebitis, along with atrophy and destruction of tissues. Immunohistochemical staining shows increased IgG4+ cells in the involved tissues (>50 per high-power field, with IgG4/IgG ratio >40%). IgG4-RSD may potentially be rarely associated with the development of lymphoma and carcinoma. However, the nature and pathogenesis of IgG4-RSD are yet to be fully elucidated and provide immense scope for further studies.

Enfermedad de Woriger kolopp diseminada: estudio innmunomorfológico / Disseminated woringer kolopp disease: immunomorphologic study

Referimos el caso de una paciente de 72 años con placas infiltradas diseminadas de bordes circinados y tendencia a la curación central.Presenta además, una lesión tumoral infiltrada y vegetante en región inguinal derecha, sin adenopatías. El estudio histológico de las placas revela un infiltrado masivo de células mononucleares, con epidermotropismo absoluto. La lesión tumoral se caracteriza por un infiltrado de células pleomórficas atípicas del dermis profundo y tejido celular subcutáneo, sin epidermotropismo. Esta lesión mostró un fenotipo a predominio de linfocitos T citotóxico (OK/8 positivo) mezclado con células reticulares /8 positivo) y a su vez con células reticulares (OK/6 positivo). Este4 fenotipo ha sido descrito en casos de micosis fungoide tumoral. Los hallazgos presentados y estudios realizados por autores reconocidos mundialmente reservan el término de enfermedad de Woringer Kolopp para la variante especial, localizada, de micosis fungoide. Es preferible clasificar el caso presentado como reticulosis pagetoide diseminada o enfermedad de Woringer Kolopp diseminada

Câncer de mama: razöes para a pesquisa rotineira da sinus histiocitose nos linfonodos axilares / Breast cancer: reasons for routine examination of sinus histiocytosis in axillary lymph nodes

Apresentam-se - fundamentadas em análise da literatura e em estudos do Autor sobre o fenômeno da sinus histiocitose (SH) nos linfonodos axilares em caso de câncer de mama - as razöes que justificam a inclusäo da SH nos laudos anatomopatológicos dos linfonodos axilares. As principais razöes säo: I. o conhecimento do grau de SH é fator importante na avaliaçäo prognóstica do caso; 2. a intensidade da SH reflete a capacidade imunológica do hospedeiro contra seu tumor; 3. a presença ou ausência de SH define subgrupos imunologicamente favorecidos ou desfavorecidos; 4. uma vez conhecidos esses subgrupos, torna-se mais fácil adotar procedimentos seletivos aproriados a cada caso individualmente. Säo feitas algumas consideraçöes em relaçäo ao tratamento adjuvante, segundo as características do subgrupo