Hodgkin Lymphoma

Epidemiology and Etiology

The incidence of Hodgkin lymphoma (HL) follows a bimodal distribution with the first peak at the age of 25 and second peak after the age of 50 years. EBV and HIV infections, autoimmune conditions, and immunosuppressant use have been described as risk factors for HL.

Pathology

  • HL is subdivided into nodular lymphocyte predominant (NLPHL) and classical HL subtypes.
  • The Reed–Sternberg (RS) cells consistently express the CD30 and CD15 antigens. In contrast to the other histologic subtypes, RS cells are infrequent in NLPHL. Instead, “popcorn cells” are seen within a background of inflammatory cells in NLPHL.

Diagnosis

Clinical Presentation

Most patients present with painless lymphadenopathy. B symptoms are more common in advanced stages.

Diagnostic Testing

FNA is often inadequate to make a diagnosis, and therefore a minimum of a core needle biopsy is necessary. If initial biopsy is nondiagnostic, excisional biopsy may be required. Additional workup includes history and physical, complete blood cell counts, chemistry, LDH, erythrocyte sedimentation rate (ESR), CT, PET, and bone marrow examination.

Staging

The Ann Arbor staging system classifies lymphomas into four stages (Table 22-10). Patients with early stage disease (stages I–II) are further stratified into favorable- and unfavorable-risk categories. Favorable risk is defined by the presence of two or less sites of disease, mediastinal width less than one-third of maximal thoracic diameter, ESR <50 mm/h (<30 mm/h with B symptoms), and absence of extranodal extension.1

Table 22-10: Ann Arbor Staging of Lymphomasa
StageDescription
IInvolvement of a single lymph node region (I) or single extralymphatic organ (IE).
IIInvolvement of ≥2 lymph node regions in the same side of the diaphragm.
IIIInvolvement of lymph node regions in both sides of the diaphragm.
IVDiffuse or disseminated involvement of one or more extralymphatic organs.

aModifying features: A, absence of B features; B, presence of B features; E, involvement of a single extranodal site contiguous or proximal to the involved nodal site; S, spleen involvement; X, bulky disease defined as lymph node ≥10 cm than one-third of mediastinum.

Treatment

  • The treatment of choice for HL is chemotherapy with ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine), with or without radiation, depending on the stage and risk category.
  • Interim PET-CT findings play an important role in guiding treatment in selected patients with HL.2
  • Relapsed disease is treated with salvage chemotherapy with or without stem cell transplantation. Brentuximab vedotin, a CD30-directed antibody–drug conjugate, and the PD-1 inhibitors pembrolizumab and nivolumab are effective treatments for relapsed HL.3,4,5

References

  1. Engert A, Plütschow A, Eich HT, et al. Reduced treatment intensity in patients with early-stage Hodgkin’s lymphoma. N Engl J Med. 2010;363:640-652. doi:10.1056/NEJMoa1000067.  [PMID:20818855]
  2. Johnson P, Federico M, Kirkwood A, et al. Adapted treatment guided by Interim PET-CT scan in advanced Hodgkin’s lymphoma. N Engl J Med. 2016;374:2419-2429. doi:10.1056/NEJMoa1510093.  [PMID:27332902]
  3. Connors JM, Jurczak W, Straus DJ, et al. Brentuximab vedotin with chemotherapy for stage III or IV Hodgkin’s lymphoma. N Engl J Med. 2018;378:331-344. doi:10.1056/NEJMoa1708984.  [PMID:29224502]
  4. Chen R, Zinzani PL, Fanale MA, et al. Phase II study of the efficacy and safety of pembrolizumab for relapsed/refractory classic Hodgkin lymphoma. J Clin Oncol. 2017;35:2125-2132. doi:10.1200/JCO.2016.72.1316.  [PMID:28441111]
  5. Ansell SM, Lesokhin AM, Borrello I, et al. PD-1 blockade with nivolumab in relapsed or refractory Hodgkin’s lymphoma. N Engl J Med. 2015;372:311-319. doi:10.1056/NEJMoa1411087.  [PMID:25482239]

Outline


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