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Lymphomas Paraneoplastic Neurological Syndromes in Hodgkin and Non-Hodgkin From bloodjournal.hematologylibrary.org by guest on July 21, 2014. For personal use only. 2014 123: 3230-3238 doi:10.1182/blood-2014-03-537506 originally published online April 4, 2014 Paraneoplastic neurological syndromes in Hodgkin and non-Hodgkin lymphomas Francesc Graus, Helena Ariño and Josep Dalmau Updated information and services can be found at: http://bloodjournal.hematologylibrary.org/content/123/21/3230.full.html Articles on similar topics can be found in the following Blood collections Free Research Articles (2531 articles) Lymphoid Neoplasia (1767 articles) Review Articles (516 articles) Information about reproducing this article in parts or in its entirety may be found online at: http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#repub_requests Information about ordering reprints may be found online at: http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#reprints Information about subscriptions and ASH membership may be found online at: http://bloodjournal.hematologylibrary.org/site/subscriptions/index.xhtml Blood (print ISSN 0006-4971, online ISSN 1528-0020), is published weekly by the American Society of Hematology, 2021 L St, NW, Suite 900, Washington DC 20036. Copyright 2011 by The American Society of Hematology; all rights reserved. From bloodjournal.hematologylibrary.org by guest on July 21, 2014. For personal use only. Review Article Paraneoplastic neurological syndromes in Hodgkin and non-Hodgkin lymphomas Francesc Graus,1,2 Helena Arin˜o,1,2 and Josep Dalmau1,3,4 1Neuroimmunology Program, Institut d’Investigacions Biom`ediquesAugust Pi i Sunyer, Barcelona, Spain; 2Department of Neurology, Hospital Clinic, Barcelona, Spain; 3Department of Neurology, University of Pennsylvania, Philadelphia, PA; and 4Instituci´o Catalana de Recerca i Estudis Avanc¸ats, Barcelona, Spain Paraneoplastic neurological syndromes PNSs such as sensory neuronopathy or the tumor itself does not trigger the PNS. (PNSs) rarely associate with Hodgkin lym- Lambert-Eaton myasthenic syndrome Third, unlike patients with solid tumors in phoma (HL) and non-HLs (NHLs). Except rarely occur in lymphomas, whereas others, patients with lymphoma, the PNSs often for paraneoplastic cerebellar degeneration such as granulomatous angiitis, are only develops at advanced stages of the dis- (PCD) in HL and dermato/ polymyositis in described in HL. Second, onconeural anti- ease. Furthermore, the type and frequency both HL and NHL, other PNSs are un- bodies are absent in most PNSs associated of PNSs are different between HL and NHL; common and have only been reported as with lymphomas with the exceptions of whereas LE and PCD occur almost exclu- isolated case reports or short series. Tr (d/notch-like epidermal growth factor- sively in patients with HL, sensorimotor neu- There are several important differences related receptor) in PCD and mGluR5 in ropathies and dermatomyositis are more in PNSs when occurring in association limbic encephalitis (LE). The antigens frequent in NHL. (Blood. 2014;123(21): with HL and NHL compared with those recognized by these antibodies are not 3230-3238) associated with solid tumors. First, some expressed in lymphoma cells, suggesting Introduction Paraneoplastic neurological syndromes (PNSs) occur with increased sensorimotor neuropathy,”“neuromyotonia,”“Lambert-Eaton myasthenic frequency in patients with cancer and are not caused by metastasis, syndrome,”“polymyositis,”“dermatomyositis,” and “myasthenia.” Articles direct infiltration of the tumor, or known indirect mechanisms such as were also identified by searches of the authors’ files. toxicity, ectopic secretion of hormones, or induced coagulopathies. When originally described, the cause of PNSs was unknown. Pres- ently, the accepted hypothesis is that many PNSs are caused by immune mechanisms triggered against antigens that are normally Diagnostic criteria of PNSs present in the nervous system and ectopically expressed by the tumor (onconeural antigens). The basis of this hypothesis is the identifi- The presence of a neurological syndrome of unclear etiology at cation of antibodies against onconeural antigens in serum and cerebral the time of the diagnosis of a tumor does not necessarily mean that the spinal fluid (CSF) of many patients with PNSs.1 neurological syndrome is paraneoplastic, as this could represent the The frequency of PNSs is low; they occur in ,1% of patients with coincidental occurrence of 2 unrelated events. In 2004, 2 levels of fi solid tumors, particularly small-cell lung carcinoma (SCLC), breast, diagnostic certainty were proposed for PNSs: de nite and possible. The criteria used to define the level are based on the type of neu- and ovarian cancers. The frequency is probably lower in Hodgkin rological syndrome, the detection of well-characterized onconeural lymphoma (HL) and other lymphomas. However, the correct diag- 2 nosis of PNS is important because an early recognition of a neu- antibodies, and the presence of a cancer (Figure 1). Some PNSs are rological syndrome as paraneoplastic often leads to the discovery and termed classical because they almost always indicate the presence of treatment of the underlying tumor, which is a crucial step in the an underlying tumor (Table 1). These syndromes are considered fi management of the PNS.1 de nite PNSs if the tumor is found or the patient has a well- characterized onconeural antibody. Nonclassical syndromes, such as sensorimotor neuropathy, would qualify as a definite PNS only if the patient has a well-characterized onconeural antibody or the syn- drome improves after successful treatment of the underlying tumor Methods (Figure 1).2 Well-characterized onconeural antibodies are those that are demonstrated with validated tests, and for which there are a References for this review were identified through searches of PubMed for number of published reports defining the specificity and sensitivity articles published in English until December 31, 2013 with the search terms fi fi “ ”“ ” “ ” of the antibody for PNS and con rmation of the ndings by several Hodgkin disease, lymphoma, in combination with Ophelia syndrome, 2 2 “limbic encephalitis,”“granulomatous angiitis,”“paraneoplastic cerebellar investigators. Since the publication of the PNS criteria in 2004, degeneration,”“paraneoplastic chorea,”“opsoclonus,”“stiff-person syn- 2 onconeural antibodies should be added to the list of well- drome,”“motor-neuron disease,”“paraneoplastic sensory neuropathy,” characterized onconeural antibodies: Sox1 antibodies which are autoimmune autonomic neuropathy and/or ganglionopathy,”“paraneoplastic markers of an underlying SCLC in patients with paraneoplastic Submitted March 10, 2014; accepted April 2, 2014. Prepublished online as © 2014 by The American Society of Hematology Blood First Edition paper, April 4, 2014; DOI 10.1182/blood-2014-03-537506. 3230 BLOOD, 22 MAY 2014 x VOLUME 123, NUMBER 21 From bloodjournal.hematologylibrary.org by guest on July 21, 2014. For personal use only. BLOOD, 22 MAY 2014 x VOLUME 123, NUMBER 21 PARANEOPLASTIC NEUROLOGICAL SYNDROMES IN LYMPHOMAS 3231 Figure 1. Flowchart showing the level of diagnostic evidence for the diagnosis of PNSs. Reprinted with permission from J Neurol Neurosurg Psychiatry 2004; 75:1135-1140.2 cerebellar degeneration (PCD) or Lambert-Eaton myasthenic 3,4 syndrome (LEMS), and Tr antibodies, which are markers of General overview of PNSs in lymphomas HL in patients with PCD.5 The Tr antigen has been recently identified as d/notch-like epidermal growth factor-related re- PNSs are rare in lymphomas. Except for PCD in HL and dermato/ 6 ceptor (DNER). polymyositis in both HL and non-HL (NHL), other PNSs have rarely The recently described antibodies against neuronal cell surface or been reported in these patients. The frequency of PNSs associated synaptic receptors are not included in the group of well-characterized with lymphomas was analyzed by the PNS Euronetwork consortium onconeural antibodies because they are excellent markers (and that includes 20 European centers. Between 2000 and 2008, the appear to be pathogenic) of the neurological syndrome, but may consortium collected 53 patients with PNSs, 29 of them with NHL 7-16 occur in patients with or without cancer (Table 2). For these cell and 24 with HL. The most commonly associated PNS was PCD in surface or synaptic antibodies, the frequency of an underlying tumor 21 patients, 16 of them with HL, and demyelinating neuropathies in varies with the type of antibody, age, and sometimes gender of 11, 9 with NHL.17 the patient; in some cases (eg, a-amino-3-hydroxy-5-methyl-4- PNSs that occur in lymphomas have several important differ- isoxazolepropionic acid[AMPA]org-aminobutyric acid B [GABAB] ences compared with those in solid tumors. First, lymphomas as- receptor antibodies), a search for an underlying tumor is indicated sociate with unique PNSs (granulomatous angiitis, hypothermia), because cancer may be present in up to 60% of the patients whereas classical PNSs (sensory neuronopathy, LEMS) rarely occur. 7 (Table 2). Second, onconeural antibodies are absent in most PNSs (Table 1); only Tr and metabotropic glutamate receptor type 5 (mGluR5) are considered good markers of an underlying HL. This is a limitation to Table 1. Paraneoplastic neurological syndromes define as definite many PNS that associate with lymphomas. Third, Associated Predominant Selected onconeural antigens are not expressed in the tumor cells, suggesting Syndrome antibodies lymphoma type references the tumor itself may not trigger the PNS.
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