Learning Outcome

1. Recall the presentation of Hodgkin lymphoma
2. Summarize the treatment of Hodgkin lymphoma
3. Discuss the complications of treatment of Hodgkin lymphoma
4. 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 into two distinct categories: classical Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma (NLP-HL). These two disease entities show differences in the clinical picture and pathology. Classical Hodgkin lymphoma accounts for approximately 95% of all HL, and it is further subdivided into four 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 lymphoma has 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 of Hodgkin lymphoma is unknown. However, there is an increased risk of Hodgkin lymphoma in Epstein-Barr (EBV) infection, autoimmune diseases, and immunosuppression. There is also evidence of familial predisposition in Hodgkin lymphoma. EBV has been found to be more common in the mixed cellularity and lymphocyte depleted subtypes of Hodgkin 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 with Hodgkin lymphoma, suggesting a gene-environment interaction role in Hodgkin lymphoma predisposition.[4][5][6]

Risk Factors

The exact etiology of Hodgkin lymphoma is unknown. However, there is an increased risk of Hodgkin lymphoma in Epstein-Barr (EBV) infection, autoimmune diseases, and immunosuppression. There is also evidence of familial predisposition in Hodgkin lymphoma. EBV has been found to be more common in the mixed cellularity and lymphocyte depleted subtypes of Hodgkin 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 with Hodgkin lymphoma, suggesting a gene-environment interaction role in Hodgkin lymphoma predisposition.[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 of Hodgkin lymphoma has 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 for Hodgkin lymphoma is 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 of Hodgkin lymphoma is 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 metabolic panel (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 of Hodgkin 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 with Hodgkin lymphoma is to cure the disease with control of short and long-term complications. There are several different staging systems for Hodgkin 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:

1. Age older than 45 years
2. Stage-IV disease
3. Male gender
4. WBC greater than 15,000/mL
5. Lymphocyte less than 600/mL
6. Albumin less than 4.0 g/dL
7. Hemoglobin less than 10.5 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 of Hodgkin lymphoma depends 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 of Hodgkin lymphoma will 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 with Hodgkin lymphoma is 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 with Hodgkin lymphoma is 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.