CD30+ Diseases: Anaplastic Large-Cell Lymphoma and Lymphomatoid Papulosis

The CD30 antigen was first detected in 1982 using a Hodgkin-Reed/Sternberg (H-RS) cell line and it was cloned and characterized as a member of the tumor necrosis factor (TNF) receptor superfamily a decade later in 1992 (1–3). Soon it became clear that, although restricted to hematopoietic cells, it was not only expressed by H-RS cells but also by activated T- and B-cells and some other lymphoid malignancies. In addition to Hodgkin’s lymphoma (HL), the following primary systemic lymphomas also express CD30: anaplastic large-cell lymphoma (ALCL) in all cases, the majority of posttransplant lymphoproliferative disorders (PTLDs, see Chapter 12), the primary mediastinal B-cell lymphoma with a comparably weak CD30 expression (see Chapter 10), and the lymphomatoid granulomatosis (Lyg) in some instances (see Chapter 11). In addition, CD30 is expressed in primary cutaneous CD30-positive T-cell lymphoproliferative disorders, to which the primary cutaneous ALCL (C-ALCL), the lymphomatoid papulosis (LyP), and the borderline lesions belong.

This chapter will focus on the systemic ALCL and the primary cutaneous CD30-positive T-cell lymphomas, although CD30 is expressed by many others of the rare lymphoid malignancies. Because CD30 is the red thread for this chapter, this receptor will be described more in detail first.