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WHO Classification of Tumours
Introduction: Hodgkin lymphomas


Definition
Hodgkin lymphomas (HL) share the following characteristics: (1) they usually arise in lymph nodes, preferentially in the cervical region; (2) the majority of them manifest clinically in young adults; (3) neoplastic tissues usually contain a small number of scattered large mononucleated and multinucleated tumour cells (designated Hodgkin and Reed-Sternberg cells or HRS cells) residing in an abundant heterogeneous admixture of non-neoplastic inflammatory and accessory cells; (4) the tumour cells are often ringed by T lymphocytes in a rosette-like manner. Hodgkin lymphomas account for ~30% of all lymphomas. Their absolute incidence has not apparently changed, in contrast with non-Hodgkin lymphomas where there has been a steady increase in incidence.

Synonyms and historical annotation
This neoplasm was recognized in the first half of the 19th century by Thomas Hodgkin

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Hodgkin (1832)
On some Morbid Appearances of the Absorbent Glands and Spleen.
Med Chir Trans 17: 68-114



and Samuel Wilks
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Wilks S (1856)
Cases of lardaceous disease and some allied affections, with remarks.
Guy's Hosp Rep 17: 103.




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Wilks S (1856)
Enlargement of the lymphatic glands and spleen (or, Hodgkin's disease) with remarks.
Guy's Hosp Rep 11: 56.



. The latter named it Hodgkin disease. The disease was also called lymphogranulomatosis, a term that is no longer in use. Since the origin of the Reed-Sternberg cell is known to be a lymphoid cell —most often of B-cell type— the term Hodgkin lymphoma is preferred over Hodgkin’s disease. The modern classification of Hodgkin lymphoma is based on the Lukes-Butler scheme
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Lukes R, Butler J, Hicks E (1966)
Natural history of Hodgkin's disease as related to its patholgical picture.
Cancer 19: 317–344.




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Lukes RJ, Craver L, Hall T, Rappaport H, Ruben P (1966)
Report of the nomenclature committee.
Cancer Res 26: 1311.



.

Subclassification
Biological and clinical studies in the last 30 years have shown that Hodgkin lymphomas are comprised of two disease entities

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Anagnostopoulos I, Hansmann ML, Franssila K, Harris M, Harris NL, Jaffe ES, Han J, van Krieken JM, Poppema S, Marafioti T, Franklin J, Sextro M, Diehl V, Stein H (2000)
European Task Force on Lymphoma project on lymphocyte predominance Hodgkin disease: histologic and immunohistologic analysis of submitted cases reveals 2 types of Hodgkin disease with a nodular growth pattern and abundant lymphocytes.
Blood 96: 1889-99




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Diehl V, Sextro M, Franklin J, Hansmann ML, Harris N, Jaffe E, Poppema S, Harris M, Franssila K, van Krieken J, Marafioti T, Anagnostopoulos I, Stein H (1999)
Clinical presentation, course, and prognostic factors in lymphocyte-predominant Hodgkin's disease and lymphocyte-rich classical Hodgkin's disease: report from the European Task Force on Lymphoma Project on Lymphocyte-Predominant Hodgkin's Disease.
J Clin Oncol 17: 776-83




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Mason DY, Banks PM, Chan J, Cleary ML, Delsol G, de Wolf Peeters C, Falini B, Gatter K, Grogan TM, Harris NL (1994)
Nodular lymphocyte predominance Hodgkin's disease. A distinct clinicopathological entity.
Am J Surg Pathol 18: 526-30




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Nogová L, Reineke T, Brillant C, Sieniawski M, Rüdiger T, Josting A, Bredenfeld H, Skripnitchenko R, Müller RP, Müller-Hermelink HK, Diehl V, Engert A, (2008)
Lymphocyte-predominant and classical Hodgkin's lymphoma: a comprehensive analysis from the German Hodgkin Study Group.
J Clin Oncol 26: 434-9



: nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) and classical Hodgkin lymphoma (CHL). These two entities differ in their clinical features and behaviour as well as in the composition of their cellular background and —diagnostically most important— in the morphology, immuno­phenotype and the preservation or extinction of B-cell gene expression program. Within classical HL, four subtypes have been distinguished: nodular sclerosis, mixed cellularity, lymphocyte-rich and lymphocyte-depleted. These subtypes differ in their sites of involvement, clinical features, growth pattern, presence of ­fibrosis, composition of cellular background, number and/or degree of atypia of the tumour cells and frequency of ­Epstein-Barr virus (EBV) infection, but not in the immunophenotype of the tumour cells, which is the same in all four variants. A detailed account of the historical evolution of the subclassification of Hodgkin lymphoma is provided by Mauch et al.
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Mauch P, Armitage JO, Diehl V
Hodgkin's Disease
Lippincott Williams & Wilkins: Philadelphia. 1999



and by Anagnostopoulos et al.
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Anagnostopoulos I, Hansmann ML, Franssila K, Harris M, Harris NL, Jaffe ES, Han J, van Krieken JM, Poppema S, Marafioti T, Franklin J, Sextro M, Diehl V, Stein H (2000)
European Task Force on Lymphoma project on lymphocyte predominance Hodgkin disease: histologic and immunohistologic analysis of submitted cases reveals 2 types of Hodgkin disease with a nodular growth pattern and abundant lymphocytes.
Blood 96: 1889-99



.

Staging
Treatment of HL is based on clinical, and occasionally on pathological staging of the disease. The modified Ann Arbor staging system

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Lister TA, Crowther D, Sutcliffe SB, Glatstein E, Canellos GP, Young RC, Rosenberg SA, Coltman CA, Tubiana M (1989)
Report of a committee convened to discuss the evaluation and staging of patients with Hodgkin's disease: Cotswolds meeting.
J Clin Oncol 7: 1630-6



is used (Table 12.01).







Harald Stein
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Harald Stein
Institute for Pathology
Campus Benjamin Franklin
Berlin
Germany