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Chapter 22: Hodgkin Lymphoma

Michael Fuchs; Volker Diehl

INTRODUCTION

Epidemiology

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Epidemiology
Incidence: 8110 Estimated new cases for 2019 in the United States; 3.0 per 100,000 males; 2.3 per 100,000 females
Deaths: Estimated 1000 in 2019
Median age: 39 years
Male to female ratio: 1.3:1
Surveillance, Epidemiology and End Results (SEER) Program, available from http://seer.cancer.gov [accessed in 2019]

Pathology

Since 1944, several classifications have been proposed for Hodgkin lymphoma (HL). Currently, the World Health Organization (WHO) Classification of Hematologic Malignancies is used:

  1. Lymphocyte predominant, nodular (NLPHL) (5%)

  2. Classic

    1. Lymphocyte-rich (LRCHL) (5%)

    2. Nodular sclerosis (NSHL) (60–80%)

    3. Mixed cellularity (MCHL) (15–30%)

    4. Lymphocyte depleted (LDHL) (1%)

    5. Unclassifiable (<1%)

Swerdlow SH et al (editors). WHO classification of tumours of haematopoietic and lymphoid tissues. In: Bosman FT, Jaffe ES, Lakhani SR, Ohgaki H (editors). World Health Organization Classification of Tumours. Lyon, France: IARC; 2008

 

Swerdlow SH et al. Blood 2016;127:2375–2390

Work-up

  1. History and physical exam

  2. Laboratory tests: CBC with differential, ESR, electrolytes, albumin, liver function tests, mineral panel, LDH

  3. HIV and hepatitis B and C serologies as clinically indicated

  4. Chest x-ray (PA and lateral)

  5. CT scan of chest, abdomen, and pelvis (and neck in selected cases)

  6. Positron emission tomography (PET) scan

  7. Bone marrow aspirate and biopsy

  8. Pulmonary function tests

  9. Echocardiogram or MUGA scan to determine cardiac ejection fraction

  10. Excisional lymph node biopsy to completely assess lymph node architecture is required at initial diagnosis. Fine-needle aspiration biopsy alone is not desirable for the initial diagnosis of lymphoma

  11. Fertility counseling, if appropriate

Five-Year Survival Rate

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Five-Year Survival Rate
NLPHL 90% (10 years)
Classic HL 70–80%
Jaffe ES et al. (editors). World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC Press; 2001

Staging

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Staging
Ann Arbor Staging Classification for Hodgkin and Non-Hodgkin Lymphomas
Stage Description
Stage Description
I Involvement of a single lymph node region (I) or involvement of a single extralymphatic organ or site (IE)
II Involvement of 2 or more lymph node regions or lymphatic structures on the same side of the diaphragm alone (II) or with involvement of limited, contiguous extralymphatic organ or tissue (IIE)
III Involvement of lymph node regions on both sides of the diaphragm (III), which may include the spleen (IIIS), or limited, contiguous extralymphatic organ or site (IIIE), or both (IIIES)
IV Diffuse or disseminated foci of involvement of one or more extralymphatic organs or tissues with or without associated lymphatic involvement
Abbreviations
A Asymptomatic
B Unexplained persistent or recurrent fever with temperature higher than 38°C (100.4°F) or recurrent drenching night sweats within 1 month or unexplained loss of >10% body weight within 6 months
E Limited direct extension into extralymphatic organ from adjacent lymph node

Carbone PP et al. Cancer Res 1971;31:1860–1861

Criteria that predict an unfavorable prognosis in limited-stage HL...

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