This is a neoplastic proliferation of lymphocytes which accumulate in lymph nodes or tissue forming a mass

– They are divided into Hodgkin’s lymphoma (characterized by Reed-Sternberg Cells) and Non-Hodgkin’s

Hodgkin Lymphoma (HL)

This is a malignant proliferation of Reed-Sternberg (RS) cells. These are large B cells with prominent nucleoli in their multilobed nuclei (owl eyed nuclei)

– The RS cells are CD15 and CD30, and secrete cytokines giving B cell symptoms

– They attract inflammatory cells which make up the tumour bulk

– It usually occurs in a bimodal distribution – seen in 20s and in 60-70-year-old patients

Non-Hodgkin’s Lymphoma

This refer to a group of lymphomas without Reed-Sternberg cells. Overall, these are more common and affects the elderly.

Risk Factors:

EBV infection, immunodeficiency (HIV), autoimmune disease, Sjogren’s syndrome

Types:

i) Follicular lymphoma

This is a proliferation of small B cells that form a follicle-like nodule

– Due to Chr 14 –> 18 translocation activating Bcl2 inhibiting apoptosis + treated with Rituximab

– This is low grade but can transform into diffuse large B cell lymphoma.

ii) Marginal zone lymphoma

This is a proliferation of B cells expanding into the lymph node marginal zone.

– It is associated with chronic inflammatory states like Hashimoto disease + H. Pylori

iii) Diffuse large B-cell lymphoma

This is a proliferation of large B cells growing in sheets

– Most common form of Non-Hodgkin’s lymphoma and very aggressive

iv) Burkitt lymphoma

This is a proliferation of medium B cells, which is most aggressive form.

– Due to c-Myc gene translocation from Chr 8 –> 14 and associated with EBV

– Histology shows “starry sky” appearance of B cells and macrophages with dead cells

– There is an African form, which usually involves the jaw –> risk factor is EBV + Plasmodium Falciparum

– There is also a sporadic form which commonly involves the abdomen, giving ileo-caecal tumours

Symptoms:

– Superficial lymphadenopathy is most common finding (but no alcohol induced pain)

– Systemic B cell symptoms –> fever, night sweats, weight loss (occur later than Hodgins’ s lymphoma)

– Extra-nodal disease (more common) –> gut + bone marrow, lungs

Diagnosis:

Blood tests – FBC shows raised WCC, LDH, inflammatory markers

Biopsy of the lymph node is diagnostic –> then CT scan to assess staging

Staging:

Ann Arbor system –> works same as for Hodgkin’s using stages 1-4

– Also add the letter A (no B cell symptoms) or B (if B cell symptoms are present) e.g. Stage 3B

Management:

– Chemotherapy and radiotherapy

– Surgery can also be used to excise large lymph nodes 

Tumour Lysis Syndrome (TLS)

This is a fatal condition which occurs after chemotherapy for high-grade hematological malignancies

– It occurs from the breakdown of the tumour cells and the subsequent release of chemicals from these cells

– This gives rise to electrolyte abnormalities + clinical features

Symptoms:

– AKI –> high urate can lead to acute tubular necrosis, giving azotemia

– Palpitations and arrhythmias –> caused by the high potassium due to AKI

– Electrolyte abnormalities –>Raised Uric Acid, Raised K+, Raised PO43-, Low Ca2+

Management:

Supportive treatment for AKI and hyperkalaemia

– The most important aspect is of prevention. Therefore, during chemotherapy cycles, patients are given prophylactic allopurinol or rasburicase (makes uric acid more easily excreted)

– Therefore it is very important to monitor uric acid levels and U&Es in lymphoma/leukaemia patients who are receiving chemotherapy