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Lymphoma - Non B cell neoplasms

Hodgkins Lymphoma – general


Reviewer: Dragos Luca, M.D. (see Reviewers page)
Revised: 27 September 2011, last major update September 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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Hodgkin lymphomas (HL) share the following characteristics:
● Usually arise in lymph nodes, more commonly cervical
● Arise in a single node or chain of nodes, spread characteristically to anatomically contiguous nodes to spleen to liver to marrow to extra-nodal
● Clinically manifest in young adults
● Have small numbers of mono- and multinucleated Hodgkin and Reed-Sternberg (HRS) cells
● Demonstrate an abundant background of mixed inflammatory and accessory cells
● Show tumor cells often ringed by T-lymphocyte rosettes

Incidence
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● ~30% of all lymphomas, 1% of all cancers
● Incidence apparently unchanged (in contrast with non-HL which has shown a steady increase in incidence)
● Common malignancy of young adults (mean age 32); also occurs after age 50; 75% are curable

Symptoms / historical annotation
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● First recognized by Thomas Hodgkin and Samuel Wilks (1st half of the 19th century)
● Named Hodgkin disease by Wilks
● Also named lymphogranulomatosis in the past
● Since HRS cells are now known to be lymphoid cells, most commonly B-cells, Hodgkin lymphoma is the preferred term

Classification
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● Based on the Lukes-Butler scheme
● Based on biological studies, there are two distinct entities: nodular lymphocyte predominant HL (NLPHL) and classical HL (CHL)
● Four subtypes of CHL: nodular sclerosis, mixed cellularity, lymphocyte-rich and lymphocyte-depleted

Staging
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● Important in determining therapy since Hodgkin lymphoma spreads in an orderly fashion

I: Involvement of a single lymph node region or lymphoid structure (e.g. Waldeyer’s ring, thymus or spleen)
IE: Localized involvement of a single extranodal site in the absence of any lymph node involvement
II: Involvement of two or more lymph node regions on the same side of the diaphragm (the mediastinum is a single site; hilar lymph nodes are lateralized)
IIE: Localized involvement of a single extranodal site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (the number of anatomic sites should be indicated by suffix, e.g. II3)
III: Involvement of lymph node regions or structures on both sides of the diaphragm (III), which also may be accompanied by extranodal extension associated with adjacent lymph node involvement (IIIE) or by involvement of the spleen (IIIS) or both (IIIE, S)
Splenic involvement is designated by the letter S
III1: With or without splenic, hilar, celiac or portal nodes
III2: With paraaortic, iliac or mesenteric nodes
IV: Diffuse or disseminated involvement of one or more extranodal sites, with or without associated lymph node involvement; or isolated extranodal involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s)
Stage IV includes any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid

References: Cotswold Revision of the Ann Arbor staging classification (J Clinical Oncol 1989;7:1630)

A = absence of B symptoms, B = presence of significant fever, night sweats, >= 10% unexplained weight loss, pruritus
X = bulky disease (1/3 the width of the mediastinum or >10 cm)

End of Lymphoma - Non B cell neoplasms > Hodgkins Lymphoma – general


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