Hodgkin's disease, in the World Health Organization (WHO) classification known as 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, 2]. They are designated classical Hodgkin's disease (classical HD) and nodular lymphocyte-predominant Hodgkin's disease (NLPHD). It is now known that both types of Hodgkin's disease are B-cell neoplasms but, because of the fairly distinctive features of this condition, subdivision of lymphoma into Hodgkin's disease and non-Hodgkin's lymphoma (NHL) has been maintained. The cell of origin in both cases is a germinal centre B cell. The differences between these two types of Hodgkin's disease are summarized in Table 24.1. Histologically Hodgkin's disease is defined by the presence of characteristic neoplastic cells (Reed-Sternberg cells and Hodgkin's cells or their variants) in a setting of inflammatory cells with or without fibrosis. Classical HD 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.
Table 24.1A comparison of features of classical and nodular lymphocyte-predominant Hodgkin's disease (HD) |Favorite Table|Download (.pdf) Table 24.1 A comparison of features of classical and nodular lymphocyte-predominant Hodgkin's disease (HD)
| ||Classical HD ||Nodular lymphocyte-predominant HD |
|Frequency ||95% of cases ||5% of cases |
|Aetiology ||Some cases associated with EBV infection ||No association with EBV infection |
|Epidemiology ||Double peak of increased incidence in young adults and in old age ||Unimodal peak of incidence in young adults |
|Histology ||Reed–Sternberg cells and mononuclear Hodgkin cells ||L&H cells (popcorn cells); nodular background |
|Immunophenotype of neoplastic cells ||CD30 positive; CD15 positive in most cases; CD20 expression weak or absent; CD45, CD79a, BCL6, immunoglobulin and epithelial membrane antigen not expressed; J chain negative; PAX5 positive; BOB1 negative; OCT2 variable but more often negative; MUM1 positive; EBV detectable in neoplastic cells in some cases ||CD30 and CD15 negative; CD20, CD45, CD79a and BCL6 positive; immunoglobulin usually expressed; epithelial membrane antigen is positive in about half of cases; J chain positive; PAX5 positive; BOB1 positive; OCT2 positive; MUM1 negative; EBV is not detected in neoplastic cells; L&H cells are ringed by CD3-positive and CD57-positive T cells |
|Nature of relapse ||Late relapses very rare; relapse is as classical HD ||Late relapses are more common; relapse may be as nodular lymphocyte-predominant HD or as diffuse large B-cell lymphoma |
Hodgkin's disease commences in a single lymphocyte in a lymph node or other organ and, in the usual case in which disease starts in a lymph node, spreads initially by lymphatics to contiguous lymph nodes. There may also be local invasion and, late in the course of the disease, spreading through the blood stream to distant ...