Provincial Health Services Authority (PHSA) improves the health of British Columbians by seeking province-wide solutions to specialized health care needs in collaboration with BC health authorities and other partners.
Revised 12 April 2013
The diagnosis of Hodgkin lymphoma is based on the recognition of Reed-Sternberg cells and/or Hodgkin cells in an appropriate cellular background in tissue sections from a lymph node or extra-lymphatic organ, such as the bone marrow, lung or bone. Fine needle aspiration biopsy is not adequate for the diagnosis of Hodgkin lymphoma. Open biopsy is required because of the need to establish progress to Hodgkin lymphoma and should be distinguished from it (see lymphoma-like conditions).
Definition of Stages
The staging system for Hodgkin lymphoma used at BCCA is based on clinical staging according to the Ann Arbor system with the additions of a definition of bulky disease. Staging laparotomy is not required.
Bulky tumour is defined as a single mass of tumour tissue 10 cm or larger in largest diameter.
Mandatory Staging Procedures
Certain tests are required only in special circumstances.
Assessment of the Liver
Only well defined non-cystic space-occupying lesions documented by CT scan or sonogram can be accepted as unequivocal evidence of liver involvement. Abnormal liver function tests with or without hepatomegaly are not firm evidence of liver involvement.
Assessment of the Spleen
Patients with sonographic or CT scan evidence of discrete non-cystic lesions in the spleen are considered to have extensive splenic involvement for treatment planning. Patients with minimal enlargement of the spleen (enlarged by scan but not palpable or only the tip palpable) are not considered to have splenic involvement.
Optimal treatment of Hodgkin lymphoma depends on the histologic stage of the disease. The following table summarizes the recommended treatment according to these factors using an algorithm depending on information determined from the recommended staging procedures above. Patients who do not have a complete response to the primary treatment noted in this table should be discussed with a medical or radiation oncologist.
Involved Node Radiotherapy (INRT)
Adopted mainly from Campbell’s papers on “INRT<5cm” = pre- and post-chemo involved lymph nodes (within post-chemo anatomical limits) + 1.5-5cm to field edge or to PTV, 
Residual Disease Radiotherapy (RDRT)
 B. A. Campbell et al., “Involved-Nodal Radiation Therapy As a Component of Combination Therapy for Limited-Stage Hodgkin's Lymphoma: A Question of Field Size,” Journal of Clinical Oncology, vol. 26, no. 32, pp. 5170–5174, Oct. 2008.
 B. A. Campbell et al., “Long-term outcomes for patients with limited stage follicular lymphoma,” Cancer, vol. 116, no. 16, pp. 3797–3806, May. 2010.
 B. A. Campbell, J. M. Connors, R. D. Gascoyne, W. J. Morris, T. Pickles, and L. H. Sehn, “Limited-stage diffuse large B-cell lymphoma treated with abbreviated systemic therapy and consolidation radiotherapy,” Cancer, vol. 118, no. 17, pp. 4156–4165, Jan. 2012.
 T. Girinsky, “Radiotherapy Recommendations for Patients with Early Stage Hodgkin's Lymphoma: Involved Node Radiation Therapy (INRT),” pp. 1–9, Mar. 2008.
 T. Girinsky et al., “The conundrum of hodgkin lymphoma nodes: To be or not to be included in the involved node radiation fields. The EORTC-GELA lymphoma group guidelines,” Radiotherapy and Oncology, vol. 88, no. 2, pp. 202–210, Aug. 2008.
 P. J. Hoskin, P. Díez, M. Williams, H. Lucraft, and M. Bayne, “Recommendations for the Use of Radiotherapy in Nodal Lymphoma,” Clinical Oncology, vol. 25, no. 1, pp. 49–58, Jan. 2013.
Standard and Experimental Treatment - Overview
>15.0 x 109/L
count <0.6 x 109/L or percent <8% of WBC
When three or more of these factors are present at diagnosis (high risk), risk of treatment failure is high and the likelihood of cure with ABVD alone is only about 70%. Therefore, patients less than 66 years of age with advanced Hodgkin lymphoma and four or more of these factors should be offered treatment with experimental treatment on a clinical trial if available. Please check with one of the chemotherapists associated with the Lymphoma Tumor Group for information about which such clinical trials are available.
Standard Treatment - Specific Details
1A, 1B or 2A
CT***x 2 then PET scan
if PET neg -> CT x 2 more cycles
if PET pos -> INRT
1A, 1B or 2A
CTx6 then CT scan and marrow biopsy if originally positive
If CR, no further treatment
If otherwise in CR but residual mass > 2 cm do PET scan
If PET neg, no further treatment
If PET pos and encompassable in a reasonable radiation volume-> RDRT
If PET pos and not encompassable in a reasonable radiation volume-> close observation or biopsy to direct further treatment on proof of persistent lymphoma
2B 3A or 3B 4A or 4B
* Bulk: low = no tumor mass >10 cm; high = any single tumour mass > = 10 cm (note: round to nearest whole cm, e.g. 9.5 cm = 10 cm but 9.4 cm = 9 cm)** Risk defined using the prognostic index described above in section 22.214.171.124*** CT=chemotherapy, ABVD**** RDRT=residual disease radiation therapy***** If 4 or more risk factors present consider enrollment on clinical trial, if available
Most patients with Hodgkin lymphoma, especially those below the age of 65 years at diagnosis, can be cured with the treatments described in this section. Most cured patients experience minimal long-term toxicity from the treatments, however, certain predictable and occasional rare and unpredictable late effects may occur and require preventive measures and/or recognition and treatment.
The following late effects of Hodgkin lymphoma or its treatment should be considered when patients are reviewed in follow-up.
10% to 30% of patients relapse depending on stage and bulk of presentation. Careful attention should be directed to lymph node sites, especially if previously involved with disease.
Neck or oropharyngeal irradiation may cause decreased salivation. Patients should have careful dental care follow-up and should make their dentist aware of the previous irradiation.
After external beam thyroid irradiation to doses sufficient to cure Hodgkin lymphoma at least 50% of patients will eventually become hypothyroid. Also, there is an increased risk of secondary thyroid carcinoma after irradiation. All patients whose TSH level becomes elevated should be treated with life-long thyroxine replacement in doses sufficient to suppress TSH levels to low normal for two purposes: to maintain euthyroidism and to minimize the risk of thyroid carcinoma by reducing overstimulation with endogenous TSH.
ABVD is not known to cause any permanent gonadal toxicity based on longterm follow-up of thousands of patients. Direct or scatter radiation to gonadal tissue may cause infertility, amenorrhea or premature menopause but this seldom occurs with the fields used for the treatment of Hodgkin lymphoma. Thus, with the current chemotherapy regimens and radiation fields used, most patients will not develop these problems. In general, after treatment, women who continue menstruating are fertile, but men require semen analysis to provide a specific answer.
Although uncommon, certain secondary neoplasms occur with increased frequency in patients who have been treated for Hodgkin lymphoma. These include acute myelogenous leukemia, thyroid, breast, lung and upper gastrointestinal carcinoma and melanoma and cervical carcinoma-in-situ. It is appropriate to screen for these neoplasms for the rest of the patient's life because they may have a lengthy induction period.
The following minimum follow-up tests and examinations should be performed on all patients after treatment of Hodgkin lymphoma. Visits should be every 3 months for 2 years, then every 6 months for 3 years, then annual. Patients should be strongly encouraged to perform careful breast and skin examination on a regular basis. Immunizations should be updated as recommended in Appendix III.
Lymph node, abdominal, thyroid, and skin examinationCBC, alkaline phosphatase
Every visit for 2 years then every other visit
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