What do you know about Hodgkin’s lymphoma?

  Hodgkin lymphoma (HL) is a distinct type of lymphoma that accounts for approximately 10-20% of all lymphomas. It includes two types of disease: nodular lymphocyte predominant HL (NLPHL) and classic HL (CHL). These two types of HL share some common features: only a few tumorigenic macrophages-Hodgkin and Reed-Sternberg (R-S) cells-are present in the lesion tissue, and the tumor cells are surrounded by a large number of reactive non-tumor cells.  The two types of HL differ in clinical features, biological behavior, morphology, immunophenotype, Ig transcription, and composition of reactive cells in the background.  CHL is subdivided into four subtypes: nodular sclerosis, mixed cell type, cell-rich type, and lymphocyte-ablative type.  These subtypes differ in terms of site of origin, clinical features, growth pattern, fibrosis, composition of background reactive cells, number of tumor cells, and frequency of EBV infection, but the immunophenotype of tumor cells is the same.  The clinicopathological features and immunophenotype of each type of HL are described as follows: Classic Hodgkin’s lymphoma (1) Nodular sclerosing type (NS): relatively common in young females, usually occurring in the neck and supraclavicular lymph nodes, often with mediastinal lymph node involvement. Some patients present with mediastinal lymph nodes as the first manifestation. The microscopic features are: thick collagen fibers separating the diseased lymph nodes as nodules of varying sizes, with visible trapped cells, and nodular changes resembling lymphadenopathy when tissue involvement of the spleen, liver, bone marrow and other organs occurs during the disease process. The nodular sclerosis type of CHL does not transform into other subtypes of CHL. (2) Mixed cell type (MC): more common, more common in male patients, often with systemic symptoms and often with higher clinical stage than patients with other subtypes. The lymph node structure is damaged to varying degrees, but in the early stage, the lesions are mainly distributed in the paracortical area of the lymph nodes, and the tumor cells are mixed with various inflammatory cells, often with EBV infection, and about 70% of the cases have R-S cells containing EBV genome. As the tumor progresses, CHL-MC may transform into lymphocyte-ablative CHL.  (3) Lymphocyte rich type (LR): It is less common and has a better prognosis. Lymphocytes are present in the lesion tissue, while tumor cells are rare. About 40% of cases are associated with EBV infection.  (4) Lymphopenic type (LD): the least common subtype of CHL, less than 5%, occurs in the elderly, with high clinical stage, often with systemic symptoms and poor prognosis. There are very few lymphocytes and a large number of R-S cells or their pleomorphic variants in the lesion tissue.  In classic Hodgkin’s lymphoma R-S cells and its variant cells are CD45-, CD30+, CD15+. CD30+ is expressed in almost all cases and CD15+ in nearly 75% of cases. The expression pattern of both is paranuclear punctiform positivity with or without cell membrane positivity, and background histiocytes and granulocytes serve as internal controls. r-S cells also usually express HLA-DR, CD25, CD40, CD138, peanut agglutinin, and fascin, but not BCL6, J-chain, and EMA. latent phase membrane protein 1 (LMP1) of EBV Expression varied according to different tissue subtypes and epidemiological factors.  Nodular lymphocyte-predominant HL (NLPHL) accounts for approximately 5% of all HL, and is relatively common in middle-aged and young men with enlarged cervical and axillary lymph nodes, while mediastinal and bone marrow involvement is rare. The tumor is prone to recurrence, but the prognosis is good. Microscopically, the lymph nodes appear as darkly stained indistinct nodular structures with a large number of small B lymphocytes and some histiocytes. Popcorn-like (L&H) cells are common in the nodules, and other cellular components such as eosinophils, neutrophils and plasma cells are rare, with little necrosis or fibrotic changes. Unlike CHL, tumor cells in NLPHL, i.e., popcorn-like cells, express B-cell differentiation antigens such as CD20, CD79a, CD45, and the germinal center-specific transcription factor BCL6, and most cases express J-chain and CD75, and about 50% express EMA. In contrast, CD15 was not expressed, and rarely CD30 was expressed, and EBV infection was lacking.