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The term diffuse large B-cell lymphoma (DLBCL) covers a rather heterogeneous group of lymphomas that are all characterized by diffuse tissue infiltration by large B-lineage lymphoma cells. Those that are related to human immunodeficiency virus (HIV) infection are dealt with separately (see Chapter 15). There are uncommon sub types, including mediastinal (thymic) large B-cell lymphoma, primary effusion-associated lymphoma and intravascular B-cell lymphoma. The disease can be primarily nodal or extra-nodal and can occur de novo or represent transformation of a lower grade non-Hodgkin's lymphoma, of nodular lymphocyte predominant Hodgkin's disease or of chronic lymphocytic leukaemia (known as Richter's syndrome).

Clinical features

Patients may present with localized or generalized lymphadenopathy (Figure 14.1) or with extra-nodal disease at a great variety of sites. In advanced disease there may be hepatomegaly, splenomegaly and involvement of central nervous system or bone marrow, with or without circulating lymphoma cells. Mediastinal large B-cell lymphoma [1] presents as a thymic mass (Figures 14.2,14.3,14.4,14.5), primary effusion lymphoma with pleural or pericardial effusion or ascites (usually in an HIV-positive patient) [2] and intravascular B-cell lymphoma with multiorgan symptoms [3].

Figure 14.1

Clinical photograph showing cervical lymphadenopathy in a patient with diffuse large B-cell lymphoma (T-cell rich B-cell lymphoma).

Figure 14.2

Pre-treatment chest radiograph in a patient with mediastinal (thymic) large B-cell lymphoma.

Figure 14.3

Post-treatment chest radiograph in a patient with mediastinal (thymic) large B-cell lymphoma (same patient as Figure 14.2).

Figure 14.4

CT scan in another patient with mediastinal (thymic) large B-cell lymphoma.

Figure 14.5

CT scan in a patient with mediastinal (thymic) large B-cell lymphoma (same patient as Figure 14.4).

Haematological and pathological features

In the minority of patients with peripheral blood involvement, the lymphoma cells have a diameter that exceeds that of three erythrocytes (Figure 14.6). They are usually pleomorphic, and may have large nucleoli and irregular or cleft nuclei [4] (Figure 14.7). Sometimes cytoplasmic basophilia is prominent and a Golgi zone may be apparent.

Figure 14.6

Peripheral blood film showing large lymphoma cells with prominent nucleoli. Romanowsky stain, x 100 objective.

Figure 14.7

Ultrastructural examination showing pleomorphic large lymphoma cells, some with irregular nuclei and some with large nucleoli. Lead nitrate and uranyl acetate stain.

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