Makindo Medical Notes

Related Subjects: |Hodgkin Lymphoma |Non Hodgkin Lymphoma |Diffuse large B-cell lymphoma |Intravascular large B-cell lymphoma |Mantle cell lymphoma |Marginal Zone Lymphoma |Gastric (MALT) Lymphoma |Primary CNS Lymphoma (PCNSL) |Burkitt's lymphoma |Follicular Lymphoma

Hodgkin Lymphoma is a highly curable disease by current treatment modalities with a reported 5-year survival of 90%. Classical Hodgkin disease carries a better prognosis than all types of non-Hodgkin lymphoma

About

• 2,000 cases per year in UK and affects males more than it does females.
• Malignant disorder of lymphoid cells in lymphoid tissue

Incidence

• 4 new cases per 100,000 per annum
• 10% of lymphomas are HL and the remained NHL
• Bimodal incidence ages 15-35 and in those over 50

Aetiology

• Commoner in immunodeficiency and autoimmune disease. EBV in Immunocompromised e.g. those with HIV
• B lymphocyte predominates.
• Affected nodes show Reed-Sternberg cells which is a giant B lymphocyte with two mirror-image nuclei with 'owl's eye' nuclei and B cell clones

Risk factors

• Age: HL is most common in two age groups: young adults (15-35 years) and older adults (over 55 years).
• Gender: Males are slightly more likely to develop HL than females.
• Family History: A family history of Hodgkin Lymphoma or other lymphomas can increase the risk.
• Epstein-Barr Virus (EBV) Infection: is associated with an increased risk of HL.
• Weakened Immune System: those with HIV/AIDS or who have undergone organ transplantation, are at higher risk.

Histology

• Nodular Sclerosis: The most common subtype, often found in young adults, particularly women. It is characterized by large, nodular masses in the lymph nodes.
• Mixed Cellularity: Common in older adults, it features a mix of different types of cells, including Reed-Sternberg cells, and is associated with HIV infection.
• Lymphocyte-Rich: A rarer subtype that has a better prognosis and is more commonly seen in men.
• Lymphocyte-Depleted: The least common subtype, often seen in older adults or those with HIV, and tends to be more aggressive.

Staging

Stage B symptoms

• Caused by cytokines from tumour
• Low-grade fever, night sweats, weight loss 10%
• These suggest a worse prognosis

Clinical

• Fatigue, Drenching night sweats, Fever, Weight loss
• Generalised itching, Breathlessness
• Bruising, Bone pain, Alcohol-induced pain Abdominal pain
• Lymphadenopathy Splenomegaly
• Form, non-fixed and non-tender lymphadenopathy
• Cervical and Mediastinal lymphadenopathy
• Lymphadenopathy painful after alcohol

Investigations

• FBC, U&E, LFTs, ESR, Ca, P, ALP, LDH, Urate
• Biopsy Lymph nodes > 1 cm for 4-6 weeks may show Reed Sternberg cell
• Bone marrow aspirate and trephine biopsy
• CT/MRI Thorax Abdomen Pelvis and neck if needed
• Positron emission tomography (PET) scanning allows more accurate staging and monitoring of response to treatment
• Echocardiogram as needed

Management: NB Adriamycin is trade name for Doxorubicin

• The prognosis for Hodgkin Lymphoma is generally very good, especially when diagnosed early. The overall five-year survival rate for HL is around 85%, and for early-stage disease, it is higher. The prognosis varies depending on factors such as the stage of the disease, the patient’s age, and the presence of other health conditions.
• For patients with limited or intermediate-stage disease (Ia or IIa) combined modality treatment consisting of brief chemotherapy followed by radiation therapy (RT) is still the standard approach, also in the case of PET guided approach.
• Advanced-stage HL is usually treated with systemic treatment; additional RT is confined to approximately 10% of patients with residual disease after systemic treatment.
• Patients diagnosed with Hodgkin Lymphoma carry a lifelong risk of transfusion-associated graft versus host disease (TA-GVHD). Where blood products are required these patients must receive only irradiated blood products for life.
• Chemotherapy
  • Patients less than 60 years may be successfully treated with either ABVD (6 cycles) Adriamycin (Doxorubicin), bleomycin, vinblastine, dacarbazine or escBEACOPP (4-6 cycles) (escalated bleomycin, etoposide, Adriamycin, cyclophosphamide, Oncovin, procarbazine, prednisone). ABVD is repeated every 28 days. This is known as one Cycle.
  • Adriamycin (Doxorubicin) causes cardiac toxicity and Bleomycin causes Lung toxicity
  • New data indicate that 6 cycles of AVD + brentuximab vedotin (BV) (with obligatory G-CSF support) represent a third opportunity with efficacy and toxicity intermediate between ABVD and eBEACOPP.
  • ABVD represents the standard of care for older HL patients who are fit enough for doxorubicin containing regimens, but patients older than 65 to 75 should not receive more than 2 cycles of bleomycin due to increased severe lung toxicity. Concomitant administration of AVD+BV is too toxic in this patient population, but interesting results can be achieved with sequential administration.
  • Immunotherapy: In cases of relapsed or refractory HL, drugs that target specific immune checkpoints (e.g., nivolumab, pembrolizumab) may be used.
• High dose chemotherapy (HDCT) followed by autologous stem cell transplantation (ASCT) represents the treatment of choice for fit patients with refractory/relapsed HL with BV and anti PD-1 antibodies proposed as options in patients failing ASCT.

References