Essay 13- Hodgkin’s lymphoma
Definition
- Lymphomas are a group of diseases caused by malignant lymphocytes that accumulate in lymph nodes and other lymphoid tissue
Etiology
- Exact etiology unknown!
- Increased risk in:
- EBV infections
- HIV infections
- Autoimmune diseases
- Immunosupression
Pathophysiology
- Reed-Sternberg cell is a neoplastic, large multinucleated cell with two mirror-image nuclei (owl eyes) within a reactive cellular background
- Derived from germinal center B cells with mutations of the IgH- variable region segment
- Pathognomonic for classical HL!
Types of HL
- Lymphocyte-predominance → proliferation of small lymphocytes, histiocytes and Reed-Sternberg cells
- Nodular-sclerosis → fibrous tissue growth in lymph nodes, Red-Sternberg cells, lymphocytes, macrophages, foci of necrosis
- Mixed-cellularity → lymphocytes, eosinophils, neutrophils, plasma cells, histiocytes, foci of necrosis, Reed- Sternberg cells
- Lymphocyte-depletion → lymph nodes with depleted lymphocytes, diffuse fibrosis, Reed-Sternberg cells and Hodgkin cells
Table 19.1 WHO (2008) classification of Hodgkin lymphoma.
Symptoms
- Painless, asymmetrical, firm and discrete enlargement of superficial lymph nodes
- Cervical
- Axillary
- Inguinal
- Splenomegaly in 50% of patients
- Mediastinal involvement- pleural effusions or SVC obstruction
- Constitutional symptoms:
- Fever
- Pruritus
- Alcohol induced pain
- Weight loss
- Profuse sweating
- Weakness
- Fatigue
- Anorexia
- Cachexia
Staging (Ann Arbor staging system)
- Stage 1- one group of LN involved
- Stage 2- two or more LN regions involved
- On the same side of the diaphragm!
- Stage 3- LN involvement on both sides of diaphragm
- Stage 4- involvement outside LN areas
- Liver, BM
- Substage A- absence of “B” symptoms!
- Substage B- presence of “B symptoms!
Diagnosis
- CBC:
- Normochromic, normocytic anemia
- Neutrophilia, eosinophilia
- Lymphopenia in advanced disease
- Raised serum LDH
- LN biopsy- an RS cell or LP cell needs to be identified within the biopsy specimen to establish a definitive diagnosis!
- Excision biopsy
- Immunophenotyping- CD15 and CD30 positive
- HIV testing
- CT
- X-rays
- PET/CT
Treatment
- Depends on stage of spread of the lymphoma
- Chemotherapy or a combination of chemo and radiotherapy
- Choice depends on stage!
- Early stage- two cycles of ABVD (Adriamycin, bleomycin, vinblastine, and dacarbazine) followed by restricted involved-field radiation therapy (IFRT)
- Advanced stage- chemotherapy with different regimens (escalated BEACOPP and Stanford V)
- Autologous S.C Transplant - Pt's w Relapse or Refractory.