Lymphoma and plasma cell neoplasms
Hodgkin lymphoma
General

Author: Fatimah Algawahmed, M.D.
Editor: Rumina Musani, M.D.
Deputy Editor Review: Debra Zynger, M.D.

Revised: 23 April 2018, last major update April 2018

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Hodgkin Lymphoma [title] "loattrfree full text"[sb] Review[ptyp] NOT non

Cite this page: Algawahmed, F. Hodgkin lymphoma - general. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomanonBhodgkins.html. Accessed July 16th, 2018.
Definition / general
  • Two major types of Hodgkin lymphoma are recognized by the WHO (2016) hematopoietic and lymphoid tumors classification, reflecting the differences in clinical presentation, behavior, morphology, phenotype and molecular features
    • Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL)
    • Classic Hodgkin lymphoma (CHL), further subclassified into four different types
      • Nodular sclerosis
      • Lymphocyte rich
      • Mixed cellularity
      • Lymphocyte depleted
Essential features
  • Lymphoid tumors most often derived from germinal center B cells but demonstrate remarkable loss of the B cell phenotype, especially the classic type (Nat Rev Cancer 2009;9:15)
  • Cervical or less commonly mediastinal, axial or inguinal lymph nodes are the most frequently affected sites; primary splenic, liver or bone marrow involvement is rare (Semin Hematol 2016;53:148)
  • Contains a small number of the characteristic neoplastic cells (Hodgkin and Reed-Sternberg cells or lymphocyte predominant cells) within a background rich in inflammatory cells
  • Over 90% are of the classic type (Mediterr J Hematol Infect Dis 2014;6:e2014040)
  • Highly curable (~80%) especially with new therapeutic regimens
Terminology
  • Named after Sir Thomas Hodgkin who microscopically described the process over 150 years ago
  • Carl Sternberg and Dorothy Reed independently described the typical diagnostic cells in 1898 and 1902
Epidemiology
Sites
  • Primary site of involvement varies by the type of Hodgkin lymphoma
  • Mediastinal and supraclavicular involvement is more likely to be CHL whereas NLPHL is less common in these sites
  • An association between intraparotid nodes and NLPHL has been described (Medicine (Baltimore) 2015;94:e987)
Etiology
Clinical features
  • Localized painless lymphadenopathy
  • Fever, night sweats and weight loss are more common in CHL than NLPHL
Case reports
Gross description
  • Lymph nodes are enlarged and encapsulated and show a fish flesh tumor on cut section
Microscopic (histologic) description
  • CHL:
    • Neoplastic cells are Hodgkin and Reed-Sternberg (HRS) cells
    • Effaced lymph node with variable number of HRS cells in a background of inflammatory cells; the components of the background vary depending on the subtype
  • NLPHL:
    • Neoplastic cells are lymphocyte predominant (LP) cells (also known as popcorn cells)
    • Totally or partially effaced lymph node with nodular or diffuse infiltrate consisting of small lymphocytes, histiocytes, epithelioid elements admixed with a small number of LP cells
Microscopic (histologic) images

Images hosted on other servers:

Popcorn cell in NLPHL

Reed-Sternberg cell

Various images

Immunohistochemistry
Staging / staging classifications
  • Lugano staging system is a modified Ann Arbor classification (Semin Hematol 2016;53:148)
    • Stage I: a single lymph node region (I) or single extralymphatic site (IE)
    • Stage II: two or more lymph node regions on the same side of the diaphragm (II) or nodal stage I or II plus contiguous involvement of an extralymphatic site (IIE)
    • Stage III: lymph node regions on both sides of the diaphragm, which may include the spleen (IIIS) or limited contiguous extralymphatic organ or site (IIIE, IIIES)
    • Stage IV: disseminated (noncontiguous) involvement of one or more extralymphatic organs
  • The following letters are added to the stage to further classify specific cases:
    • A: absence of systemic symptoms
    • B: presence of systemic symptoms
    • E: localized extranodal extension from another site
    • S: splenic involvement
    • X: bulky disease
Board review question #1
Which one of the following subtypes of Hodgkin lymphoma is more prevalent in young children than adults?

  1. Lymphocyte depleted CHL
  2. Lymphocyte rich CHL
  3. Mixed cellularity CHL
  4. NLPHL
  5. Nodular sclerosis CHL
Board review answer #1
C. Mixed cellularity CHL. Mixed cellularity CHL is more common in young children, whereas nodular sclerosis CHL is more common in adolescents and young adults.
Board review question #2
Which of the following is a typical immunophenotype in Hodgkin Lymphoma?

  1. HRS cells are CD20-, CD79a- and PAX5-
  2. HRS cells are CD20-, CD79a- and PAX5+
  3. HRS cells are CD20+, CD79a- and PAX5-
  4. LP cells are CD20+, CD79a- and PAX5+
  5. LP cells are CD20+, CD79a+ and PAX5-
Board review answer #2
B. HRS cells are CD20-, CD79- and PAX5+. CD20 and CD79a are negative in the majority of cases whereas PAX5 is often expressed but it is less intense compared to reactive B cells. On the other hand, LP cells are almost always positive for all the three markers: CD20, CD79a and PAX5.