Clinical Features

LBL has a peak incidence in the second and third decades. Characteristic presenting features include male predominance, mediastinal involvement, occurring in 60-70% of patients, reflecting the thymic origin of the malignant cells in LBL, and pleural and peri-cardial effusions, sometimes with resulting cardiac tamponade. Symptoms and signs of superior vena caval obstruction may be present. Mediastinal masses are uncommon in patients with B-cell LBL. Peripheral lymph node involvement is present in 60-80% of the patients at diagnosis, most commonly in cervical, supraclavicular, and axillary regions.

LBL commonly involves the bone marrow and central nervous system (CNS). The frequency of bone marrow involvement at presentation is difficult to determine from published series in view of the variable distinction between LBL and ALL. In a recent prospective study from Europe, 21% of the adult patients with LBL had bone marrow involvement at presentation.7 Leukemic overspill is also common, but the frequency is obscured by inconsistent distinction between LBL and ALL.

CNS involvement is uncommon at presentation, occurring in approximately 5-10% of the patients. Typical manifestations of CNS involvement include meningeal involvement with a pleocytosis in the cerebrospinal fluid, or cranial nerve involvement, characteristically involving ophthalmic or facial nerves. Several reports suggest that CNS involvement at presentation is more common in patients with bone marrow involvement. Although CNS involvement at presentation is uncommon, it is a frequent site of relapse in the absence of adequate prophylaxis, when the incidence of relapse has been reported to be as high as 31%.8 Other less common sites of involvement include the liver, spleen, and subdiaphragmatic lymph nodes, as well as bone, skin, and testes.

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