Paraneoplastic Manifestations of Lymphoproliferative Neoplasms

• Published in: Lymphoma and Chronic Lymphocytic Leukemias 2016-08, Vol.2016 (2016), p.21-33
• Main Authors: Tandra, Pavankumar, Kallam, Avyakta, Guduru, Mounika, Bendi, Venkata Sunil, Krishnamurthy, Jairam, Bierman, Philip
• Format: Article
• Language: English
• Subjects: Care and treatment; Complications and side effects; Development and progression; Lymphoproliferative disorders; Paraneoplastic syndromes
• ISSN: 1179-2361; 1179-2361
• DOI: 10.4137/LCLL.S38507

Paraneoplastic syndromes, although rare, have been associated with lymphoproliferative disorders. Hodgkin's lymphoma (HL) is the most common lymphoid neoplasm known to cause paraneoplastic syndromes. These syndromes can often be the earliest manifestation of the underlying malignancy, and treating the underlying lymphoma can cure the paraneoplastic disease. Paraneoplastic diseases reported in lymphoid malignancies can be broadly classified into hematological, neurological, and dermatological syndromes based on the organ systems that are predominantly involved. In addition, renal and hepatobiliary involvement has also been reported. The pathogenesis of the hematological paraneoplastic conditions primarily involves the production of autoantibodies by the neoplastic lymphocytes, which then subsequently leads to cytopenias. Cytoses are a result of cytokine produced by the neoplasms. The administration of corticosteroids along with chemotherapy for the underlying malignancy is the treatment of choice. Neurological paraneoplastic phenomena have been reported in both HL and non-Hodgkin's lymphoma (NHL). They are thought to be secondary to an immune-related process that is triggered by the underlying process. Both the central and the peripheral nervous systems can be affected. Often, treatment of the underlying malignancy with chemotherapy can result in reversal of the paraneoplastic syndrome. In peripheral neuropathies, muscle relaxants and analgesics are often used for symptomatic relief. Dermatological manifestations, pemphigus vulgaris in particular, often precedes the malignancy. Renal and hepatobiliary manifestations, although rare, are also associated with lymphomas.