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Introduction

Hodgkin lymphoma is a histologically defined disorder. The term encompasses two distinct types of disease, which differ in aetiology, epidemiology, clinical features, pathology and prognosis [1–3]. They are designated classical Hodgkin lymphoma (classical HL) and nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL). Both types of Hodgkin lymphoma are mature B-cell neoplasms at a germinal centre stage of differentiation. Despite of the absence of expression of B-cell markers with the exception of PAX5, the Reed–Sternberg cells have clonally rearranged immunoglobulin (IG) chain genes. However, because of the fairly distinctive features of this condition, subdivision of lymphoma into Hodgkin lymphoma and non-Hodgkin lymphoma (NHL) has been maintained. The differences between the two types of Hodgkin lymphoma are summarized in Table 28.1. Histologically Hodgkin lymphoma is defined by the presence of characteristic neoplastic cells (Reed–Sternberg cells and Hodgkin cells or their variants) in a setting of inflammatory cells with or without fibrosis. Often, the neoplastic cells are ringed by non-neoplastic T lymphocytes forming a rosette. Classical HL is further subdivided into lymphocyte-rich, mixed cellularity, nodular sclerosis (or nodular sclerosing) and lymphocyte-depleted subtypes on the basis of the ratio between neoplastic cells and reactive cells, the specific cytological features of the neoplastic cells and the presence or absence of fibrous bands. Although there is variability in the frequency of these subtypes in relation to geographical regions, nodular sclerosis is the most common whilst lymphocyte depleted is the rarest.

Table 28.1A comparison of features of classical and nodular lymphocyte-predominant Hodgkin lymphoma (HL)

Hodgkin lymphoma commences in a single lymphocyte in a lymph node or other organ and, in the ...

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