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WHO Classification of Tumours
Classical Hodgkin lymphoma


Classical Hodgkin lymphoma (CHL) is a monoclonal lymphoid neoplasm probably derived from germinal center B cells. It is histologically characterized by mononuclear Hodgkin and multinucleated Reed-Sternberg (HRS) cells in a variable infiltrate of non-neoplastic small lymphocytes, eosinophils, neutrophils, histiocytes, plasma cells, fibroblasts and collagen
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Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008

CHL accounts for 95% od all Hodgkin lymphomas and has age peaks at 15-35 years and in late life. It involves cervical lymph nodes in approximately 75% of cases and patients typically present with localized disease (stages I and II). Primary extranodal involvement is rare. Current therapy for Hodgkin lymphoma has resulted in an excellent outcome and cure for up to 90% of patients.
RHS cells carry clonal immunoglobin (IG) gene rearrangements and show frequent infection with Epstein-Barr virus (EBV). CHL is a hallmark of X-linked lymphoproliferative disease (XLP) , chracterized by extreme sensitivity to EBV infection.

> Classical Hodgkin lymphoma variants
Nodular sclerosis classical Hodgkin lymphoma
Lymphocyte-rich classical Hodgkin lymphoma
Mixed cellularity classical Hodgkin lymphoma
Lymphocyte-depleted classical Hodgkin lymphoma

Gastric CHL
CHL may involve the gastrointestinal tract, but this is usually secondary to nodal disease. Primary gastric classical Hodgkin lymphoma is extremely rare

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Zippel K, Hoksch B, Zieren HU (1997)
[A rare stomach tumor--Hodgkin's lymphoma of the stomach].
Chirurg 68: 540-2

, and should be differentiated from EBV-positive DLBCL of the elderly
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Asano N, Yamamoto K, Tamaru J, Oyama T, Ishida F, Ohshima K, Yoshino T, Nakamura N, Mori S, Yoshie O, Shimoyama Y, Morishima Y, Kinoshita T, Nakamura S (2009)
Age-related Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorders: comparison with EBV-positive classic Hodgkin lymphoma in elderly patients.
Blood 113: 2629-36

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Cohen JI, Kimura H, Nakamura S, Ko YH, Jaffe ES (2009)
Epstein-Barr virus-associated lymphoproliferative disease in non-immunocompromised hosts: a status report and summary of an international meeting, 8-9 September 2008.
Ann Oncol 20: 1472-82

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Oyama T, Yamamoto K, Asano N, Oshiro A, Suzuki R, Kagami Y, Morishima Y, Takeuchi K, Izumo T, Mori S, Ohshima K, Suzumiya J, Nakamura N, Abe M, Ichimura K, Sato Y, Yoshino T, Naoe T, Shimoyama Y, Kamiya Y, Kinoshita T, Nakamura S (2007)
Age-related EBV-associated B-cell lymphoproliferative disorders constitute a distinct clinicopathologic group: a study of 96 patients.
Clin Cancer Res 13: 5124-32

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Park S, Lee J, Ko YH, Han A, Jun HJ, Lee SC, Hwang IG, Park YH, Ahn JS, Jung CW, Kim K, Ahn YC, Kang WK, Park K, Kim WS (2007)
The impact of Epstein-Barr virus status on clinical outcome in diffuse large B-cell lymphoma.
Blood 110: 972-8

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Yoshino T, Nakamura S, Matsuno Y, Ochiai A, Yokoi T, Kitadai Y, Suzumiya J, Tobinai K, Kobayashi Y, Oda I, Mera K, Ohtsu A, Ishikura S (2006)
Epstein-Barr virus involvement is a predictive factor for the resistance to chemoradiotherapy of gastric diffuse large B-cell lymphoma.
Cancer Sci 97: 163-6

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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010