Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant clonal plasma cell disorder. It is characterized by the presence of a monoclonal (M) protein in the serum or urine that is commonly discovered during laboratory work-up of a variety disorders.…
T-cell lymphomas are a highly heterogeneous group of diseases sharing common origin from mature T-lymphocytes. Heterogeneity is appreciated on biologic and clinical grounds, both in terms of presentation (leukemic, nodal, extranodal, and cutaneous forms) and clinical behavior (aggressive versus indolent).…
Malignant lymphomas are the third most common malignancy among children and adolescents. Among children less than 15 years of age, non-Hodgkin Lymphoma (NHL) is more frequent; however, in patients up to 18 years of age, Hodgkin lymphoma (HL) is predominant.…
There are five well-characterized human viruses that are generally accepted as important in lymphomagenesis ( Table 87.1 ). These viruses may infect tumor cells (or their progenitors) or may act at a distance. The genomes of Epstein-Barr virus (EBV), Kaposi…
Mantle cell lymphoma (MCL) is a distinct subtype of mature B-cell non-Hodgkin lymphoma (NHL) that accounts for 5% to 10% of all NHL. Although a subset of patients with MCL may have an indolent course, MCL is generally an aggressive…
High-grade lymphomas are aggressive hematopoietic malignancies derived predominantly from transformed B cells. Notable subtypes include diffuse large B-cell lymphoma (DLBCL), high-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements (HGBCL), and Burkitt lymphoma (BL). Although these entities share many…
Diffuse large B-cell lymphoma (DLBCL) of the central nervous system (CNS) accounts for an overwhelming majority (95%) of cases of primary CNS lymphoma (PCNSL). According to the 2016 report of the World Health Organization (WHO), it is defined as DLBCL…
The term marginal zone refers to a histologic compartment located at the periphery of lymphoid follicles immediately outside their mantle zone. The marginal zone is especially evident in the spleen, although identical areas have been observed in other lymphoid structures,…
Non-Hodgkin lymphoma (NHL) refers to all malignancies of the lymphoid system with the exception of Hodgkin lymphoma. Development of the lymphoid system is a highly regulated process, characterized by differential expression of a number of cell-surface and intracytoplasmic proteins and…
Over the past two decades, discoveries in basic immunology and the pathogenesis of malignancies have significantly advanced our understanding of the origin of lymphoid neoplasms. These diseases have been reexamined and grouped based on recurrent chromosomal rearrangements, histologic patterns, gene…