9650/3 Hodgkin lymphoma, NOS


Synonyms

Hodgkin disease, NOS
Malignant lymphoma, Hodgkin

Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C81.9
           

/span>lassical Hodgkin lymphoma (CHL) is a monoclonal lymphoid neoplasm (in most instances derived from B cells) composed of mononuclear Hodgkin cells and multinucleated Reed-Sternberg (HRS) cells residing in an infiltrate containing a variable mixture of non-neoplastic small lymphocytes, eosinophils, neutrophils, histiocytes, plasma cells, fibroblasts and collagen ?fibres. Based on the characteristics of the reactive infiltrate and to a certain extent on the morphology of the HRS cells (i.e. lacunar cells), four histological subtypes have been distinguished: lymphocyte-rich classical Hodgkin lymphoma(LRCHL), nodular sclerosis classical Hodgkin lymphoma(NSCHL), mixed cellularity classical Hodgkin lymphoma(MCCHL) and lymphocyte-depleted classical Hodgkin lymphoma (LDCHL). The immunophenotypic and genetic ?features of the mononuclear and multinucleated cells are identical in these histological subtypes, whereas their clinical features and association with Epstein-Barr virus (EBV) show differences.

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Thymus
ICD-O-3 topography code: C37
ICD10: C81.9
           

/span>lassical Hodgkin lymphoma (CHL) is a monoclonal lymphoid neoplasm probly derived from germinal center B cells. It is histologically charcterized 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
2
 
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



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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.

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Stomach
ICD-O-3 topography code: C16
ICD10: C16
           

/span>lassical 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
3
 
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.

> 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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Click to access Pubmed
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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Click to access Pubmed
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



8
Click to access Pubmed
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



9
Click to access Pubmed
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



10
 
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



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