Clinical Pearls & Morning Reports
Published September 6, 2017
Diffuse large-B-cell lymphoma is a non-Hodgkin’s lymphoma with an aggressive appearance on pathological testing. Read the new Clinical Problem Solving Article.
Q: Diffuse large-B-cell lymphoma accounts for what percentage of cases of non-Hodgkin’s lymphoma?
A: Approximately 35% of adult patients with non-Hodgkin’s lymphoma have diffuse large-B-cell lymphoma.
Q: Can diffuse large-B-cell lymphoma present with extranodal disease?
A: Approximately 40% of patients with diffuse large-B-cell lymphoma present with lymph-node involvement alone; another 40% have extranodal disease, including in the gastrointestinal tract, skin, neurologic system, bone, testis, soft tissue, salivary gland, female genital tract, lung, kidney, or liver.
A: Approximately 4% of patients with diffuse large-B-cell lymphoma have neurologic complications, most commonly leptomeningeal disease or parenchymal lesions. Peripheral neuropathy occurs in fewer than 1% of patients. It is typically due to either paraneoplastic antibodies (more common with indolent lymphomas) or direct invasion of nerves or nerve roots, known as neurolymphomatosis (more common in patients with diffuse large-B-cell lymphoma). Neurolymphomatosis usually manifests as a very painful neuropathy and may mimic the signs and symptoms of a nonmalignant, symmetric, or asymmetric peripheral neuropathy, including chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), vasculitic neuropathy, or infectious neuropathy. In patients with diffuse large-B-cell lymphoma in whom neurolymphomatosis develops at any point in the course of their disease, painful peripheral neuropathy is an uncommon initial symptom (reported in 26% of patients).
A: The diagnosis of neurolymphomatosis is more difficult to make than the diagnosis of leptomeningeal lymphomatosis. Positive cytologic studies of cerebrospinal fluid (CSF) have been reported in 21% of patients, as compared with 97% of those with leptomeningeal involvement. The sensitivities of nerve biopsy and magnetic resonance imaging (MRI) are also low. Imaging and CSF analysis often need to be repeated with the evolution of the disease in order to make the correct diagnosis. There is no high-quality evidence to guide the treatment of neurolymphomatosis — only case series of heterogeneous chemotherapeutic regimens. The median survival is 10 months.