Abstract
Malignant lymphomas are neoplasms derived from clonal proliferations of lymphocytes, comprising a diverse group of diseases, with more than 30 different subtypes defined by the “World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues” (Chap. 1) (Swerdlow et al (2008) WHO classification of tumours of haematopoietic and lymphoid tissues, 4th edn. IARC Press, Lyon).Both nodal and extranodal forms occur. They can be divided into two major groups: Hodgkin’s lymphoma (HL) and non-Hodgkin’s lymphoma (NHL). The NHLs are a large heterogeneous group of neoplasms, which can be further subdivided into those arising from B-lymphocytes or their precursors (80 %), from T-cells (14 %), and from natural killer cells (6 %). The above-mentioned WHO lymphoma classification is used for the subtyping of HL and of NHL: It emphasizes an approach whereby the clinical characteristics are correlated with distinct morphological, immunophenotypical, and genotypical features of each neoplasm (Swerdlow et al (2008) WHO classification of tumours of haematopoietic and lymphoid tissues, 4th edn. IARC Press, Lyon).For each lymphoma entity, a putative cell of origin is postulated (Chap. 1). Ocular lymphomas can be divided into intraocular lymphomas and ocular adnexal lymphomas. Intraocular lymphomas are rare with the most common type being the vitreoretinal lymphoma (VRL) (previously called “primary intraocular lymphoma”), which is usually a diffuse large B-cell lymphoma (DLBCL) occurring in the vitreous and retina, and with frequent involvement of the central nervous system (CNS) (Chan Clin Lymphoma 4:30–31, 2003; Coupland and Damato Clin Experiment Ophthalmol 36564–578, 2008; Davis Eye (Lond) 27:153–162, 2013).Other intraocular lymphomas are those arising as primary or secondary tumors in the choroid, ciliary body, and iris (Coupland and Damato Clin Experiment Ophthalmol 36564–578, 2008).
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Suggested Reading
Chan CC (2003a) Primary intraocular lymphoma: clinical features, diagnosis, and treatment. Clin Lymphoma 4(1):30–31
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Coupland, S.E. (2014). Ocular and Adnexal Lymphoma: Histopathology. In: Singh, A. (eds) Ocular and Adnexal Lymphoma. Essentials in Ophthalmology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-38499-8_2
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