Amyloidosis is defined as the clinical syndrome associated with deposition of amyloid. Amyloid in tissue is defined by its tinctorial properties of a homogeneous, eosinophilic, hyaline material when viewed by hematoxylin and eosin staining. Amyloid stains specifically with Congo red,…
Waldenström macroglobulinemia (WM) is a lymphoid neoplasm resulting from the accumulation, predominantly in the bone marrow (BM), of a clonal population of lymphocytes, lymphoplasmacytic cells, and plasma cells, which secrete a monoclonal immunoglobulin (Ig)M. WM corresponds to lymphoplasmacytic lymphoma (LPL)…
Clonal plasma cell (PC) neoplasms are comprised of a number of interrelated and partially overlapping entities, ranging from benign to fully malignant, which are variously defined and categorized by burden of disease, presence of organ injury, anatomic location, and in…
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…