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Chapter 60: Hodgkin Lymphoma

DEFINITION

  • Hodgkin lymphoma (HL) is a lymphoid neoplasm consisting of two distinct entities: classic Hodgkin lymphoma (cHL), accounting for 95% of cases, and nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL), accounting for about 5% of cases.

  • Both types of HL are derived from germinal center B cells.

  • cHL is defined by the presence of the Reed-Sternberg (RS) cells or its mononuclear variant Hodgkin cells with a characteristic immunophenotype and appropriate cellular background (Figures 60–1 and 60–2).

  • The neoplastic cells account for only 1% to 5% of cells in an affected node or tissue.

  • HL cells secrete cytokines and chemokines, which attract multiple reactive cell types, with T cells predominating. The survival of HL cells is dependent on these cells in the microenvironment.

  • cHL consists of four histologic subtypes that are distinguished based on microscopic appearance and relative proportions of RS cells, lymphocytes, and fibrosis:

    — Nodular sclerosis (~60%–65% of cases) predominates in young adults. The RS cells appear as lacunar cells.

    — Mixed cellularity (~25%–35% of cases) predominates in older adults.

    — Lymphocyte-rich (~5% of cases) is most frequent in males.

    — Lymphocyte-depleted is very rare.

  • The malignant cells in NLPHL are called lymphocytic/histiocytic cells or “popcorn” cells and are embedded in B-cell–rich nodules.

  • The distinguishing pathologic features between cHL and NLPHL are shown in Table 60–1.

FIGURE 60–1

High magnification of lymph node section in a patient with Hodgkin lymphoma. A Reed-Sternberg cell is in the center of the field with the classical findings of giant size compared to background lymphocytes, binucleation, and prominent eosinophilic nucleoli.

A photomicrograph of a stained lymph node section shows a Reed Sternberg cell, which appears as a giant cell with a bilobed nucleus and nucleoli resembling owl's eye.
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FIGURE 60–2

Classic Hodgkin lymphoma stained with antibody to CD30. CD30 stains the Reed-Sternberg cells in this lymph node biopsy.

A photomicrograph of a stained lymph node section shows multiple Reed Sternberg cells, which are C D 30 positive and stained brown.
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TABLE 60–1PATHOLOGIC FEATURES OF CLASSIC HODGKIN LYMPHOMA AND NODULAR LYMPHOCYTE-PREDOMINANT HODGKIN LYMPHOMA
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TABLE 60–1 PATHOLOGIC FEATURES OF CLASSIC HODGKIN LYMPHOMA AND NODULAR LYMPHOCYTE-PREDOMINANT HODGKIN LYMPHOMA
  Classic Hodgkin Lymphoma Nodular Lymphocyte-Predominant Hodgkin Lymphoma
Lymph node architecture Diffuse or nodular Nodular
Malignant cells Hodgkin and Reed-Sternberg cells Lymphocyte-predominant “popcorn” cells
Background cells Lymphocytes, histiocytes, neutrophils, eosinophils, plasma cells, fibroblasts Lymphocytes, histiocytes, T-cell rosettes around popcorn cells
Fibrosis Common Rare
CD30 + (>95%) Rare
CD15 + (70%–80%) Rare
CD20 Rare + (>95%)
PAX5 Dim Moderate/bright
OCT2 –/+ +
BOB.1 –/+ +
EBER Common (30%–40%) Rare

EBER, Epstein-Barr virus–encoded small RNAs.

EPIDEMIOLOGY

  • The incidence of HL is 2 to 3/100,000 per annum in the United States and western Europe.

  • The rate has been stable for several decades.

  • In 2017 in the United States, there were 8260 cases of HL.

  • An increased risk of cHL has been reported in young adults with high socioeconomic status, and the influence of environmental factors is controversial.

  • There is a bimodal age distribution, with a ...

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