Initial Presentation

DLBCL may occur in all age groups, but the incidence increases with age. The median age at presentation is 63 years, with a slight male preponderance.4 Children with DLBCL comprise only 700-800 cases per year in the United States, and tend to present with widespread disease, high-grade disease, and frequent extranodal involvement.5 Most adult patients with DLBCL also present with symptoms. Symptoms may include a rapidly enlarging lymph node, B symptoms (fevers, night sweats, >10% loss of body weight) or other constitutional symptoms, or paraneoplastic syndromes. Approximately 30-40% of the patients have an extranodal site of involvement,3 4 and DLBCL can occur in nearly every body site. Several sites merit their own diagnostic categories in the WHO classification due to unique clinical and/or pathologic features. These include primary effusion lymphoma (PEL), intravascu-lar DLBCL, and primary mediastinal lymphoma (PMBL); PMBL is discussed later. PEL and intravascular large B-cell lymphoma are extremely rare subtypes with a uniformly poor prognosis.6 PEL typically occurs in the setting of immunodeficiency and has been clearly linked to human herpesvirus 8 (HHV-8) infection. No mass can be identified, and the malignant cells instead accumulate in body cavities such as the pleural, peritoneal, or pericardial spaces. Intravascular diffuse large B-cell lymphoma is characterized by widely disseminated circulating malignant cells that frequently occlude small vessels. Diagnosis is often delayed due to heterogeneous presentations, and the pathologic features are often not appreciated until the time of autopsy.

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