Lymphoma

Lymphoma is cancer of lymphatic cells, typically presenting as a solid tumour.

Signs and symptoms include:

• night sweats
• fever
• weight loss
• fatigue
• palpable node

Diagnosis is by lymph node biopsy.

Other investigations include:

• CXR 
• Staging CT 
• Bloods – FBC, ESR, CRP, LDH, LFTs 

The classical division of lymphoma is into:

• Hodgkins lymphoma
• characteristic Reed-Sternberg cell
• around 25% of lymphomas
• males >females
• Non-Hodgkin lymphomas

More detailed classification of lymphoma is fiendishly complex; the current system in use is the WHO classification, which divides lymphomas into B cell and T cell.  A classification which is arguably more useful for 'basic' doctors is the one below which divides lymphomas into 'low grade/indolent' and 'high grade/aggressive'

Indolent:

• Hodgkin lymphoma - excellent prognosis, often cured
• Non-Hodgkins 
• Lymphoma of mucosa-assoicated lymphoid tissue (MALT)
• Follicular lymphoma 

Aggressive: (all are non-Hodgkins)

• Diffuse large B cell lymphoma (=30-60% of non-Hodgkins lymphomas) 
• Mantle cell lymphoma
• Primary effusion lymphoma 
• Burkitt lymphoma 

Staging:

• I: single lymph-node region or single extralymphatic site
• II: two or more lymph node regions or sites but on same side of diaphragm
• III: involvement of lymph nodes/regions on both sides of diaphragm
• IV: disseminated
• A or B: 
• A: no B symptoms
• B: fever, weight loss or night sweats

Increased risk of lymphoma with: 

• Exposure to pesticides 
• Immune deficiencies 
• Common variably immune deficiency (lifetime risk 8%) 
• Wiscott-Aldrich syndrome 
• Severe combined immune deficiency 
• Infections
• HIV - Burkitt lymphoma, primary effusion lymphoma, DLBCL
• EBV – Burkitt lymphoma 
• Herpes virus 8 - primary effusion lymphoma 
• Helicobacter pylori – MALT
• Campylobacter jejuni – small bowel lymphoma 
• Medications 
• Metotrexate – DLBCL, Hodgkins 
• Infliximab – T cell lymphoma 

Treatment: 

• Hodgkins lymphoma:
• ABVD chemotherapy (=adriamycine, bleomycin, vinvlastine and dacarbazine 
• Radiotherapy
• Exact treatment is based on a combination of the Ann Arbor staging and presence/absence of
• 3 involved lymph node areas 
• high ESR 
• large mediastinal mass 
• extranodal disease
• High-grade Non-Hodgkins lymphoma 
• CHOP chemotherapy (= cyclophosphamide, doxorubicin, vincristine, prednisone) 
• rituxumab
• Low-grade Non-Hodgkins lymphoma
• multiple options ranging from 'watch and wait' to chlorambucil to CHOP and rituximad.
 

References:

Dave, S. Host factors for risk and survival in lymphoma. Hematology Am Soc Hematol Educ Program. 2010; 2010:255-258. 

Engert, A. et al. Hodgkin’s lymphoma: ESMO Clinical recommendations for diagnosis, treatment and follow-up. Ann Oncol. 2009; 20(Suppl 4): 108-109. 
Gribben, J. How I treat indolent lymphoma. Blood. 2007; 109(11): 4617-4626. 

Jaffe, E. The 2008 WHO classification of lymphomas: implications for clinical practice and translational research. ASH Education Book January 1, 2009 vol. 2009 no. 1 523-531.
NICE. Non-Hodgki’s lymphoma – rituximad: guidance. 2003. Available at http://guidance.nice.org.uk/TA65/Guidance/pdf/English
OMIM: Hodgkin's disease. Available at http://omim.org/entry/236000?search=hodgkin&highlight=hodgkin

Taylor, P. et al. Hodgkin's disease: a population-adjusted clinical epidemiology study (PACE) of management at presentation. Northern Region Lymphoma Group. QJM. 1998; 91(2):131-9.

Tily, H. et al. Diffuse large B-cell Non-Hodgkin’s lymphoma: ESMO Clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2010; 21(Suppl 5): 172-174.