Anaplastic Lymphoma Kinase Positive Large B-Cell Lymphoma: Diagnostic Perils and Pitfalls, an Underrecognized Entity

Abstract

Anaplastic lymphoma kinase (ALK) positive large B-cell lymphoma (ALK+ LBCL) is an extremely uncommon non-Hodgkin lymphoma (NHL) with a distinctive histomorphologic, immunophenotypic and cytogenetic profile. It is unlike the more common ALK-positive anaplastic large cell lymphoma, although the latter shares the ALK rearrangement pathognomonic for this entity. ALK+ LBCL is an underrecognized entity since it is rare and unfamiliar, and shares morphologic and immunohistochemical features with a variety of other neoplasms that can result in misdiagnosis. This lymphoma exhibits plasmacytoid morphology and negativity for classical immunomarkers of B- and T-cell lineages, and CD30; however, it expresses terminally differentiated B-cell/plasma cell markers such as CD38, CD138, and MUM-1. Precise identification of this entity is pivotal because of its aggressive behaviour, poor response to standard chemotherapy regimens and the potential for the development of novel targeted therapy. A high index of suspicion on morphology and an extensive immunohistochemistry armoury are required for the veracious detection of this lymphoma, especially at extranodal sites. The purpose of bringing forth this present case, an extranodal neoplasm with plasmacytoid morphology at vertebral location in a young adult, is to highlight the diagnostic perils and pitfalls, the clues to unravel the quandaries and thus, the incredible utility of histopathological examination and immunohistochemical analysis in attaining the unerring diagnosis.