Morphology and immunophenotype Characteristically this malignancy shows large cells with plasmablastic morphology involving the subcapsular sinus of lymph nodes. As indicated by the
name, these lymphomas are positive for ALK in a finely granular cytoplasmic pattern (58). This cytoplasmic pattern supports the function of CLTC in moving ALK expression from the cell membrane. Furthermore, this neoplasm shows variable CD30 expression, with frequent CD38 and CD138 co-expression in the absence of earlier B cell antigens such as CD19, CD20, CD22, CD79a and CD79b (57). Distinction of this entity from plasmablastic Inhibitors,research,lifescience,medical lymphoma is largely based on ALK positivity and lack of Epstein-Barr early ribonucleoprotein 1 (EBER1) expression, which are typical features of ALK-positive large B cell lymphoma (58). Molecular abnormality The presence of t[2;17] [p23;q23] has been suggested as a genetic mechanism for ALK-positive B cell lymphoma, including cases that occur in the GI tract. The translocation joins clathrin (CLTC) and ALK, resulting in a fusion gene Inhibitors,research,lifescience,medical (57,58). Prognosis In the more commonly encountered CD30-positive anaplastic large cell lymphoma of T-cell or null type, ALK expression is generally regarded as a good prognostic Inhibitors,research,lifescience,medical factor. In ALK-positive
large B-cell lymphoma, however, only four cases have been fully characterized, with a documented median survival of 11 months in patients with stages III-IV disease (59). Lymphomatoid granulomatosis (LG) Lymphomatoid granulomatosis is an extranodal angiodestructive disease composed of EBV-positive B cells within a dominant background reactive T-cell population.
It most commonly occurs in the lung; however, the gastrointestinal tract may rarely Inhibitors,research,lifescience,medical be involved (60). Pathogenesis EBV has been hypothesized to play a role in disease pathogenesis. As such, immunocompromised patients are at increased risk in developing this lesion. Morphology and immunophenotype LG is an angiocentric/angiodestructive infiltrate of polymorphous lymphocytes. Inhibitors,research,lifescience,medical Admixed plasma cells and histiocytes are usually observed, however, neutrophils and eosinophils are not typically conspicuous. Invasion of vascular walls by lymphocytes may lead to adjacent necrosis due to compromised vascular integrity. Distinction between the similarly angiodestructive sinonasal NK/T-cell lymphoma is learn more paramount and can be accomplished by immunophenotypic analysis. LG typically consists of mature, CD20+ B cell population, frequently co-expressing EBV-encoded found RNA, in a background of reactive, CD4+ and CD8+ T cells (60). Molecular abnormalities Molecular techniques detect both clonality of immunoglobulin genes and presence of EBV-encoded RNA. Key to the differential of NK/T-cell lymphomas, T cell receptor (TCR) gene rearrangement analysis will show germline configuration in true NK-cell lesion (i.e., no evidence of monoclonality) (60). Prognosis Typically the disease is aggressive, with median survival falling below two years.