Lymphoma and Myeloma

Lymphoma

Lymphoma is the proliferation of lymphoid cells primarily in lymph nodes and other lymphoid tissue (as opposed to leukaemia which is expansion in the bone marrow). 60% of proliferation occurs in nodal tissue and 40% in extranodal tissue. Lymphoma can be low and high grade. Low grade lymphoma typically occurs in the old, whereas high grade tends to occur to the young.

Lymphoma produces immune incompetence. Additionally, immune incompetence can predispose to the disease. There is an association with certain micro-organisms, including EBV (Hodgkins and Burkitt's Lymphoma), hepatitis C, HHV8 (Kaposi's sarcoma), and Helicobacter pylori.

The disease is traditionally dividided in to Hodgkins (15% of lymphoma) and non-Hodgkins (85% of lymphoma) disease.

Hodgkins Lymphoma

This occurs in two age groups: young males, and those over 55. It presents as painless lymphadenopathy (although there may be pain on drinking alcohol), splenomegaly, fatigue, and the'B symptoms' which comprise weight loss, fever and night sweats. There may be dyspnoea due to pressure of lymph nodes on the trachea.

Investigations include:

1. Lymph node biopsy: Reed-Sternberg cells (have an 'owls eye' appearance) are pathognomic. The biopsy is staged as: nodular sclerosing, lymphocyte poor, mixed, and lymphocyte predominant.
2. Plasma viscosity: raised due to 'paraprotein' produced in excess from the lymphoid cells.
3. Paraprotein: abnormal immunoglobulin
4. CXR: large lymph nodes may be seen
5. CT abdomen: staging

The patient is staged according to the following system:

I	II	III	IV
Single region of nodes affected	2 sites the same side of the diaphragm	Several sites both sides of the diaphragm	Non-lymphatic spread (eg bone marrow)

The patients are further divided in to those asymptomatic (eg IA), and those with the 'B symtoms' listed above (eg IVB).

The disease can be curable.Treatment depends on staging. IA-2A require chemotherapy and radiotherapy. 2B-4B only have chemotherapy. The ABVD (Adriamycin, bleomycin, vinblastine, and dacarbazine) regime is the standard. Splenectomy is no longer performed with spleen involvement.

Non-Hodgkins Lymphoma

This is more commonly disseminated disease at multiple sites, and non-lymphatic infiltration. Presentation is similar to Hodgkins lymphoma with a painless swelling of a group of lymph nodes. The disease can be low or high grade.

Low grade

This involves more mature/well differentiated cells. The disease is slowly progressing and responds well tro treatment. However, relapse is common and the disease often transforms in to high grade. The mean survival is 7-10 years. Treatment involves chemotherapy including chlorambucil. Monoclonal antibody therapy (to antigens on B lymphocytes) can be tried. Radiotherapy is employed if the disease is limited to a localised site.

High grade

This disease is aggressive and rapidly progressive. Treatment is with chemotherapy (cyclophosphamide, adriamycin, vincristine, prednisolone - the CHOP regime). Bone marrow transplantation may be suitable.

Myeloma

This is the uncontrolled proliferation of plasma cells. It affects the middle-aged and elderly. The prognosis is generally poor with a survival of 3-5 years post diagnosis.

Myeloma affects a number of organ systems, which gives rise to its typical symptoms:

• Marrow infiltation: anaemia
• Low immunoglobulin: infection
• Skeletal disease: pain, fractures, hypercalcaemia
• Renal failure: damage due to paraprotein
• Peripheral neuropathy: praprotein damage
• Hyperviscosity syndrome: seizures/clouding of consciousness

The typical presentation might be an elderly lady with chronic back pain with anaemia and a raised plasma viscosity

Investigations comprise:

1. FBC: anaemia, increased viscosity
2. Blood film: "Rouleaux" may be seen
3. Calcium: raised
4. U+Es: hyperuricaemia
5. Paraprotein electrophoresis of blood
6. Urine sample for Bence Jones protein
7. Bone marrow: > 10% plasma cells
8. Skeletal survey/MRI: lytic lesions

General treatment includes managing infections, renal failure, giving allopurinol for hyperuricaemia, plasmophoresis for hyperviscosity.

Specific chemotherapy includes 'High Dose Therapy' (vincristine, adriamycin, dexamethasone) followed by peripheral cstem cell autograft. If HDT is not suitable (eg the patient is too old) then melphalan and prednisolone is delivered. Thalidomide is now used in the treatment of myeloma.