Learning Outcome
Recall the presentation of Hodgkin lymphoma
Summarize the treatment of Hodgkin lymphoma
Discuss the complications of treatment of Hodgkin lymphoma
List the nurse's role in managing patients with Hodgkin lymphoma
Introduction
Hodgkin lymphoma (HL), formerly called Hodgkin's disease, is a rare monoclonal lymphoid neoplasm with high cure rates. Biological and clinical studies have divided this disease entity intotwo distinct categories: classicalHodgkin lymphomaand nodular lymphocyte-predominant Hodgkin lymphoma (NLP-HL). Thesetwo disease entities show differences in the clinical picture and pathology. ClassicalHodgkin lymphomaaccounts for approximately 95% of all HL, and it is further subdivided intofour subgroups: nodular sclerosis (NSHL), lymphocyte-rich (LRHL), mixed cellularity (MCHL), and lymphocyte-depleted (LDHL). Four features characterize Hodgkin lymphomas.They commonly arise in the cervical lymph nodes; the disease is more common in young adults; there are scattered large mononuclear Hodgkin and multinucleated cells (Reed-Sternberg) intermixed in a background of a mixture of non-neoplastic inflammatory cells; finally, T lymphocytes are often observed surrounding the characteristic neoplastic cells.Hodgkin lymphomahas an excellent overall prognosis with approximately an 80% cure rate.[1][2][3]
Nursing Diagnosis
Weight loss
Anorexia
Fever
Itching
Risk for infection related to impaired primary and secondary defenses
Chest heaviness
Anxiety
Fatigue
Causes
The exact etiology ofHodgkin lymphomais unknown. However, there is an increased risk ofHodgkin lymphomain Epstein-Barr (EBV) infection, autoimmune diseases, and immunosuppression. There is also evidence of familial predisposition inHodgkin lymphoma. EBV has been found to be more common in the mixed cellularity and lymphocyte depleted subtypes ofHodgkin lymphoma. Loss of immune surveillance has been proposed as the possible disease etiology in EBV-positive disease. No other virus has been found to play a major contributing role in disease pathogenesis. Immunosuppression secondary to a solid organ or hematopoietic cell transplantation, therapy with immunosuppressive drugs, and human immunodeficiency (HIV) infection have all a higher risk of developing Hodgkin lymphoma. HIV patients commonly present with a more advanced stage, unusual lymph nodes sites, and a poor prognosis. Studies found that there is a ten-fold increase of developing HL in same-sex siblings of patients withHodgkin lymphoma,suggesting a gene-environment interaction role inHodgkin lymphomapredisposition.[4][5][6]
Risk Factors
The exact etiology ofHodgkin lymphomais unknown. However, there is an increased risk ofHodgkin lymphomain Epstein-Barr (EBV) infection, autoimmune diseases, and immunosuppression. There is also evidence of familial predisposition inHodgkin lymphoma. EBV has been found to be more common in the mixed cellularity and lymphocyte depleted subtypes ofHodgkin lymphoma. Loss of immune surveillance has been proposed as the possible disease etiology in EBV-positive disease. No other virus has been found to play a major contributing role in disease pathogenesis. Immunosuppression secondary to a solid organ or hematopoietic cell transplantation, therapy with immunosuppressive drugs, and human immunodeficiency (HIV) infection have all a higher risk of developing Hodgkin lymphoma. HIV patients commonly present with a more advanced stage, unusual lymph nodes sites, and a poor prognosis. Studies found that there is a ten-fold increase of developing HL in same-sex siblings of patients withHodgkin lymphoma,suggesting a gene-environment interaction role inHodgkin lymphomapredisposition.[4][5][6]
Assessment
Patients with Hodgkin lymphoma frequently present with painless supra-diaphragmatic lymphadenopathy (one to two lymph node areas), B symptoms including unexplained profound weight loss, high fevers, and drenching night sweats. B symptoms are evident in up to 30% of patients and are generally more common in stage 3 to 4 of the disease, mixed cellularity, and lymphocyte depleted HL subtypes. Pain in lymph nodes may occur with alcohol consumption (paraneoplastic symptom). Chronic pruritus is another disease symptom that may be encountered. If mediastinal nodes enlargement is significant, the mass effect can produce chest pain and shortness of breath. If the patient has an extra-nodal disease, which is less common, related clinical manifestations may occur.
Each subtype ofHodgkin lymphomahas distinct clinical features. Nodular sclerosis subtype affects young adults and presents with early disease stage, while mixed cellularity HL is prevalent in both children and elderly patients and commonly presents with advanced disease stage. Lymphocyte depletion HL presents with extensive extranodal disease, affects elderly patients, and is associated with AIDS infection. Lymphocyte-rich classical HL presents with localized painless peripheral lymphadenopathy similar to NLP-HL. NLP-HL is a distinct, unique clinicopathological entity that is distinct from classical HL. It presents in males with localized painless peripheral lymphadenopathy in the neck that often spares the mediastinum. NLP-HL shows a more indolent course with a tendency for late relapses.[7][8][9]
Evaluation
Definitive diagnosis forHodgkin lymphomais through biopsy from a lymph node or suspected organ. It is important to note that fine-needle aspiration or core-needle biopsy frequently show non-specific findings because of the low ratio of malignant cells and loss of architectural information. So excisional biopsy should be pursued if suspicion ofHodgkin lymphomais high. An RS cell or LP cell need to be identified within the biopsy specimen to establish a definitive diagnosis. Further workup is essential to determine the stage, which guides treatment and provide prognostic information.
Laboratory tests include complete blood count (CBC), complete metabolicpanel (CMP), erythrocyte sedimentation rate, Hepatitis B virus, hepatitis C virus, and HIV.
LDH levels correlate with the bulk of disease. Elevated levels of alkaline phosphatase may suggest liver or bone involvement. Testing for HIV is recommended as the treatment of the infection can improve outcomes in HIV positive individuals.
Chest x-ray, CT chest/abdomen/pelvis, and PET/CT scans can help with staging. PET-CT scanning has now become a standard test for assessment of treatment response in HL and most lymphomas. Overall, a comprehensive workup is essential for both diagnosis and staging ofHodgkin lymphoma.
Medical Management
Treatment of Hodgkin lymphoma largely depends on the histologic characteristics, the stage of the disease, and the presence or absence of prognostic factors. The goal of treatment for patients withHodgkin lymphomais to cure the disease with control of short and long-term complications. There are several different staging systems forHodgkin lymphoma, and the Cotswolds modified Ann Arbor classification is commonly used. The International Prognostic Factors Project on Advanced Hodgkin's lymphoma identified 7 variables for patients with advanced disease:
Age older than 45 years
Stage-IV disease
Male gender
WBC greater than 15,000/mL
Lymphocyte less than 600/mL
Albumin less than 4.0 g/dL
Hemoglobin less than10.5 g/dL
Risk stratification categorizes patients as low risk or high risk for recurrence. The response to therapy is determined by PET scan and is used to optimize therapy. The initial treatment ofHodgkin lymphomadepends on subgroup treatment. There are three treatment subgroups: patients with the early-stage disease with favorable prognostic factors, patients with the limited-stage disease who have unfavorable prognostic factors, and those with advanced-stage disease. Patients who are in early-stage (stage I to IIA) with favorable prognostic features are treated with short duration of chemotherapy, typically two cycles of ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) followed by restricted involved-field radiation therapy (IFRT).
Patients who are in limited-stage disease but with unfavorable features such as bulky mediastinal disease, elevated ESR, and extra-nodal extension are treated with a longer course of chemotherapy (4 to 6 cycles) followed by a higher dose of IFRT. Patients with advanced-stage (stage IIB to IV) are risk-stratified by a different scoring system, the International Prognostic Score (IPS). Depending on IPS, different chemotherapy regimens (for example, escalated BEACOPP and Stanford V) can be used, but the standard of care is ABVD for most patients.
Radiation, in general, is not beneficial in these patients. Despite the high cure rate with initial therapy, approximately 10% of patients with Hodgkin lymphoma are refractory to initial treatment, and up to 30% of patients will relapse after achieving an initial complete remission. High-dose chemotherapy, followed by an autologous stem cell transplant is the standard of care for the majority of patients who are refractory or relapse post-initial therapy. For patients who fail autologous transplantation treatment options include: brentuximab vedotin, PD-1 blockade, non-myeloablative allogeneic transplantation, or clinical trials. Future directions in the management ofHodgkin lymphomawill include the incorporation of frontline therapeutics that have shown efficacy in refractory/relapses disease setting as well as other possible novel therapeutics.[10][11][12][13]
Hematopoietic stem cell transplantation is done in refractory or relapsed patients.
Nursing Management
Vitals
Educate the patient about the disease
Weigh patient
Encourage nutrition
Monitor breathing and oxygen saturation
Not changes in color
Monitor for respiratory distress
Manage pain
Provide comfort measures
Prevent skin breakdown
When To Seek Help
Fever
Hypotension
Change in skin color
Unresponsive
Not eating
Intense pain
Outcome Identification
Able to eat
Normal vitals
No breathing difficulty
Monitoring
Vitals
Oxygenation
Nutrition
Skin integrity
Bodyweight
Intake and output
Lung function and breathing
Coordination of Care
Hodgkin lymphoma is a systemic disorder that is best managed by an interprofessional team for best outcomes.
The management of Hodgkin lymphoma is primarily by the oncologists. However, the patient may first present to the primary care provider or nurse practitioner with symptoms suggestive of the lymphoma. The key is prompt referral so that therapy can be initiated.
Treatment of Hodgkin lymphoma largely depends on the histologic characteristics, the stage of the disease, and the presence or absence of prognostic factors. The goal of treatment for patients withHodgkin lymphomais to cure the disease with control of short and long-term complications.
The pharmacist has to educate the patient on the drugs, their benefits, and side effect profile. In addition, the pharmacist has to ensure that the patient has had the recommended preoperative workup before dispensing the drugs. The oncology nurse should monitor the patient for acute side effects of the chemotherapeutic drugs and educate the patient on minimizing complications.
Because many patients develop anxiety and depression, a mental health provider should provide appropriate counseling.
The dietitian should be involved in educating the patient on foods to eat and what to avoid.
The interprofessional team has to meet on a weekly basis to discuss patient care and future therapy. The communication between the members should be clear and open to ensure that the patient's treatment has not been jeopardized. Finally, if the patient is terminal, a hospice care team should be involved.
Outcomes
Prognosis depends on several prognostic factors, including disease stage. Disease stage is currently only one factor in the prognostic indices used for pretreatment risk stratification and assessment. The 5-year overall survival (OS) in stage 1 or 2a is approximately 90%; on the other hand, stage 4 disease has a 5-year OS of approximately 60%.[14][15](Level 5)
Health Teaching and Health Promotion
Do not smoke
Avoid alcohol
Wash hands
Wash foods
Get immunized
Avoid crowds during treatment
Follow up with oncologist
Eat healthy
Exercise
Discharge Planning
Do not smoke
Avoid alcohol
Wash hands
Wash foods
Get immunized
Avoid crowds during treatment
Follow up with oncologist
Eat healthy
Exercise
Pearls and Other issues
Hodgkin lymphoma is a systemic disorder that is best managed by an interprofessional team for best outcomes.
The management of Hodgkin lymphoma is primarily by the oncologists. However, the patient may first present to the primary care provider or nurse practitioner with symptoms suggestive of the lymphoma. The key is prompt referral so that therapy can be initiated.
Treatment of Hodgkin lymphoma largely depends on the histologic characteristics, the stage of the disease, and the presence or absence of prognostic factors. The goal of treatment for patients withHodgkin lymphomais to cure the disease with control of short and long-term complications.
The pharmacist has to educate the patient on the drugs, their benefits and side effect profile. In addition, the pharmacist has to ensure that the patient has had the recommended preoperative workup before dispensing the drugs. The oncology nurse should monitor the patient for acute side effects of the chemotherapeutic drugs and educate the patient on minimizing complications.
Because many patients develop anxiety and depression, a mental health nurse should provide appropriate counseling.
The dietitian should be involved in educating the patient on foods to eat and what to avoid.
The interprofessional team has to meet on a weekly basis to discuss patient care and future therapy. The communication between the members should be clear and open to ensure that the patient's treatment has not been jeopardized. Finally, if the patient is terminal a hospice care team should be involved.
Figure
Reed Sternberg Cells Lymphoma Image Courtesy S Bhimji MD
References
- 1.
Lees C, Keane C, Gandhi MK, Gunawardana J. Biology and therapy of primary mediastinal B-cell lymphoma: current status and future directions. Br J Haematol. 2019 Apr;185(1):25-41. [PMC free article: PMC6594147] [PubMed: 30740662]
- 2.
Amraee A, Evazi MR, Shakeri M, Roozbeh N, Ghazanfarpour M, Ghorbani M, Ansari J, Darvish L. Efficacy of nivolumab as checkpoint inhibitor drug on survival rate of patients with relapsed/refractory classical Hodgkin lymphoma: a meta-analysis of prospective clinical study. Clin Transl Oncol. 2019 Aug;21(8):1093-1103. [PubMed: 30739242]
- 3.
Metzger ML, Mauz-Körholz C. Epidemiology, outcome, targeted agents and immunotherapy in adolescent and young adult non-Hodgkin and Hodgkinlymphoma. Br J Haematol. 2019 Jun;185(6):1142-1157. [PubMed: 30729493]
- 4.
PDQ Pediatric Treatment Editorial Board. PDQ Cancer Information Summaries [Internet]. National Cancer Institute (US); Bethesda (MD): Dec 18, 2023. Childhood Hodgkin Lymphoma Treatment (PDQ®): Health Professional Version. [PubMed: 26389170]
- 5.
Milgrom SA, Elhalawani H, Lee J, Wang Q, Mohamed ASR, Dabaja BS, Pinnix CC, Gunther JR, Court L, Rao A, Fuller CD, Akhtari M, Aristophanous M, Mawlawi O, Chuang HH, Sulman EP, Lee HJ, Hagemeister FB, Oki Y, Fanale M, Smith GL. A PET Radiomics Model to Predict Refractory Mediastinal Hodgkin Lymphoma. Sci Rep. 2019 Feb 04;9(1):1322. [PMC free article: PMC6361903] [PubMed: 30718585]
- 6.
Lyapichev KA, You MJ. Unusual presentation of classic Hodgkin lymphoma. Blood. 2019 Jan 31;133(5):502. [PubMed: 30705049]
- 7.
Justiz Vaillant AA, Stang CM. StatPearls [Internet]. StatPearls Publishing; Treasure Island (FL): Aug 14, 2023. Lymphoproliferative Disorders. [PubMed: 30725847]
- 8.
Gaut D, Schiller GJ. Hematopoietic stem cell transplantation in primary central nervous system lymphoma: a review of the literature. Int J Hematol. 2019 Mar;109(3):260-277. [PubMed: 30671909]
- 9.
Cai Q, Fang Y, Young KH. Primary Central Nervous System Lymphoma: Molecular Pathogenesis and Advances in Treatment. Transl Oncol. 2019 Mar;12(3):523-538. [PMC free article: PMC6371000] [PubMed: 30616219]
- 10.
Hoppe RT, Advani RH, Ai WZ, Ambinder RF, Aoun P, Armand P, Bello CM, Benitez CM, Bierman PJ, Chen R, Dabaja B, Dean R, Forero A, Gordon LI, Hernandez-Ilizaliturri FJ, Hochberg EP, Huang J, Johnston PB, Kaminski MS, Kenkre VP, Khan N, Maddocks K, Maloney DG, Metzger M, Moore JO, Morgan D, Moskowitz CH, Mulroney C, Rabinovitch R, Seropian S, Tao R, Winter JN, Yahalom J, Burns JL, Ogba N. NCCN Guidelines Insights: Hodgkin Lymphoma, Version 1.2018. J Natl Compr Canc Netw. 2018 Mar;16(3):245-254. [PubMed: 29523663]
- 11.
Gordon LI. Strategies for Management of Relapsed or Refractory Hodgkin Lymphoma. J Natl Compr Canc Netw. 2017 May;15(5S):716-718. [PubMed: 28515253]
- 12.
Klimm B, Goergen H, Fuchs M, von Tresckow B, Böll B, Meissner J, Glunz A, Diehl V, Eich HT, Engert A, Borchmann P. Impact of risk factors on outcomes in early-stage Hodgkin's lymphoma: an analysis of international staging definitions. Ann Oncol. 2013 Dec;24(12):3070-6. [PubMed: 24148816]
- 13.
Hoppe RT, Advani RH, Ai WZ, Ambinder RF, Aoun P, Bello CM, Bierman PJ, Blum KA, Chen R, Dabaja B, Duron Y, Forero A, Gordon LI, Hernandez-Ilizaliturri FJ, Hochberg EP, Maloney DG, Mansur D, Mauch PM, Metzger M, Moore JO, Morgan D, Moskowitz CH, Poppe M, Pro B, Winter JN, Yahalom J, Sundar H., National Comprehensive Cancer Network. Hodgkin lymphoma, version 2.2012 featured updates to the NCCN guidelines. J Natl Compr Canc Netw. 2012 May;10(5):589-97. [PubMed: 22570290]
- 14.
Chihara D, Oki Y, Fanale MA, Westin JR, Nastoupil LJ, Neelapu S, Fayad L, Fowler NH, Cheah CY. Stage I non-Hodgkin lymphoma: no plateau in disease-specific survival ? Ann Hematol. 2019 May;98(5):1169-1176. [PubMed: 30617643]
- 15.
Ramchandren R, Advani RH, Ansell SM, Bartlett NL, Chen R, Connors JM, Feldman T, Forero-Torres A, Friedberg JW, Gopal AK, Gordon LI, Kuruvilla J, Savage KJ, Younes A, Engley G, Manley TJ, Fenton K, Straus DJ. Brentuximab Vedotin plus Chemotherapy in North American Subjects with Newly Diagnosed Stage III or IV Hodgkin Lymphoma. Clin Cancer Res. 2019 Mar 15;25(6):1718-1726. [PubMed: 30617130]
Disclosure: Hatem Kaseb declares no relevant financial relationships with ineligible companies.
Disclosure: Hani Babiker declares no relevant financial relationships with ineligible companies.
Disclosure: Chaddie Doerr declares no relevant financial relationships with ineligible companies.
