Pathologic And Prognostic Features

Over the past several decades, DLBCL is increasingly understood to be a heterogenous disease with widely variable clinical outcomes. The Ann Arbor staging system (Table 54.1) provides a measure of tumor burden, and is an essential, though no longer sufficient, determinant of prognosis and treatment. Approximately 40% of patients present with localized disease (stage I and II) and the rest have disseminated disease at initial presentation.15 In general, the prognosis of early-stage patients is better than that of patients with advanced disease. Still, the prognosis of individual patients is not accurately predicted by the Ann Arbor stage alone. In 1993, an international task force developed a model, called the International Prognostic Index (IPI), that can be easily implemented for individual patients, using readily available clinical, laboratory, and radiographic features.15 This study analyzed over 2000 patients receiving anthracycline-based treatment for clinical features potentially predictive of outcome and identified five that retained independent prognostic

Table 54.1 Ann Arbor (Cotswold revision) staging system for malignant lymphomas

Ann Arbor staging for malignant lymphomas

Stage I

Involvement of a single lymph node region or a lymph node structure of a single extralymphatic site

Stage II

Involvement of two or more lymph node regions on the same side of the diaphragm or localized contiguous involvement of an extralymphatic site and lymph node organ

Stage III

Involvement of lymph node regions on both sides of the diaphragm

Stage IV

Diffuse or disseminated involvement of one or more extranodal organs or tissues, with or without associated lymph node involvement

A: absence of B symptoms

B: presence of B symptoms (fevers, night sweats, weight loss > 10% of body weight)

E: extranodal disease or extension from known nodal site of disease X: bulkv disease (> 1/3 widening of the mediastinum at T5-T6 or maximum size of nodal mass > 10 cm)

A: absence of B symptoms

B: presence of B symptoms (fevers, night sweats, weight loss > 10% of body weight)

E: extranodal disease or extension from known nodal site of disease X: bulkv disease (> 1/3 widening of the mediastinum at T5-T6 or maximum size of nodal mass > 10 cm)

Table 54.2 The International Prognostic Index

Adverse factor

Risk group

Number of factors present

5-year DFS (%)

5-year OS (%)

Age > 60 years

Low

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