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
Histology
- Nodular sclerosis: 70%: Good prognosis
- Mixed cellularity:25%: Good prognosis. Seen with HIV
- Lymphocyte rich :5%: Best prognosis
- Lymphocyte depleted: rare : Worst prognosis
Staging
Staging | Description |
I | Single node group or extralymphatic |
II | Involvement of two or more lymph nodes or extralymphatic site on one side of diaphragm |
III | Involvement of two or more lymph nodes or extralymphatic site or spleen on both sides of diaphragm |
IV | Diffuse Involvement of extra nodal tissue e.g. marrow or lung |
A | No systemic symptoms |
B | Weight loss > 10% or drenching night sweats |
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 Thorax Abdomen Pelvis and neck of 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
- 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.
- 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