Reactive lymphadenopathies that mimic lymphoma: Entities of unknown etiology

Division of Hematopathology, University of Texas/M.D. Anderson Cancer Center, Houston, Texas. Electronic address: .
Seminars in Diagnostic Pathology (Impact Factor: 2.56). 03/2013; 30(2). DOI: 10.1053/j.semdp.2012.08.007
Source: PubMed


Kikuchi-Fujimoto disease, Kimura disease, Rosai-Dorfman disease and IgG4 related lymphadenopathy may present with enlarging masses clinically mimicking lymphoma. A combination of clinical and histologic findings is necessary to diagnose these important rare entities, which may occasionally have aggressive clinical behavior. The recognition of these disorders is important in order to avoid misdiagnosis of malignancy, other systemic diseases such as systemic lupus, and to institute correct management and therapy, such as steroid treatment for IgG4 related lymphadenopathy. The underlying etiologies of these diseases are not completely clear at present, however, their recognition has become more common as diagnostic techniques improve. Their diagnosis and recognition may help to elucidate their underlying pathobiology.

Download full-text


Available from: Dennis P O'Malley
  • Source

    Full-text · Article · Jan 2014 · The Journal of pediatrics
  • [Show abstract] [Hide abstract]
    ABSTRACT: The head and neck region is a common site for extranodal lymphomas, second only to the gastrointestinal tract; and 12% to 15% of all head and neck tumors are lymphomas. Non-Hodgkin lymphomas are most common, and Hodgkin lymphoma occurs rarely at extranodal sites in the head and neck. Most non-Hodgkin lymphomas of the head and neck region are of B-cell lineage, and the Waldeyer ring is the most common site. Head and neck lymphomas have distinctive epidemiological and clinicopathologic features, including an association with immunosuppression, infectious organisms, or autoimmune disorders; site-specific differences (eg, thyroid gland versus ocular adnexa) for common lymphomas, such as extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue; and genetic differences that provide insights into etiology. Furthermore, the diagnosis of non-Hodgkin lymphomas at extranodal sites implies differences in prognosis and therapeutic implications with lymphomas at nodal sites. In this review, we discuss various types of non-Hodgkin lymphomas and Hodgkin lymphoma, focusing on unique aspects related to the head and neck region. We also discuss a number of newer entities that are clinically indolent as well as mimics of lymphoma that can occur in the head and neck region, including infectious mononucleosis, Kikuchi-Fujimoto disease, Kimura disease, Castleman disease, and immunoglobulin G4-related disease. Copyright © 2015. Published by Elsevier Inc.
    No preview · Article · Jun 2015 · Human pathology
  • [Show abstract] [Hide abstract]
    ABSTRACT: Since the earliest reports in 2001, immunoglobulin G4 (IgG4)-related disease has been defined as an autoimmune systemic disease characterized by the lymphoplasmacytic infiltration of affected tissues leading to fibrosis and obliterative phlebitis along with elevated serum IgG4 levels. Prior to this unifying hypothesis, a plethora of clinical manifestations were considered as separate entities despite the similar laboratory profile. The pathology can be observed in virtually all organs and may thus be a challenging diagnosis, especially when the adequate clinical suspicion is not present or when obtaining a tissue biopsy is not feasible. Nonetheless, the most frequently involved organs are the pancreas and exocrine glands but these may be spared. Immunosuppressants lead to a prompt clinical response in virtually all cases and prevent histological sequellae and, as a consequence, an early differential diagnosis from other conditions, particularly infections and cancer, as well as an early treatment should be pursued. We describe herein two cases in which atypical disease manifestations were observed, i.e. one with recurrent neck lymph node enlargement and proptosis, and one with jaundice. Our understanding of the pathogenesis of IgG4-related disease is largely incomplete but data support a significant role for Th2 cytokines with the contribution of innate immunity factors such as toll-like receptors, macrophages and basophils. Further, macrophages activated by IL4 overexpress B cell activating factors and contribute to chronic inflammation and the development of fibrosis. We cannot rule out the possibility that the largely variable disease phenotypes reflect different pathogenetic mechanisms and the tissue microenvironment may then contribute to the organ involvement. Published by Elsevier B.V.
    No preview · Article · Jun 2015 · Autoimmunity reviews
Show more