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490 Chapter 12: Haematology and clinical immunology

or tumour suppressor genes. Multiple chromosomal involves CT scan of the abdomen and a chest X-ray or
translocations have been identified. Other factors in- CT.
clude viruses (Epstein-Barr virus particularly associated
with Burkitt lymphoma), enviromental factors, congen- Management
ital and acquired immunodeficiency and some autoim- Treatment is dependent on the nature and distribution
mune conditions. Helicobacter pylori is associated with of the lymphoma. Available options include chemother-
primary gastrointestinal lymphomas. apy, radiotherapy and monoclonal antibody treatment.
The role of bone marrow transplantation is under inves-
tigation.
Clinical features
Indolent: Most patients present with painless slowly

Prognosis
progressive lymphadenopathy. Lymph nodes may re-
Indolent lymphomas have a predicted median survival
duce in size spontaneously making it difficult to dis-
time of 5–10 years. Aggressive lymphomas are more re-
tinguish from reactive lymphadenopathy. B symp-
sponsive to chemotherapy but have a predicted median
toms (fever >38 C, drenching night sweats, weight
◦
survival 2–5 years.
loss of more than 10% within 6 months) are not
common at presentation. Bone marrow failure leads
to anaemia, recurrent infections and bleeding. On Paraproteinaemias
examination there is lymphadenopathy and hep-
atosplenomegaly.
Aggressive lymphomas: Most patients present with
Multiple myeloma
lymphadenopathy, extranodal involvement (gas-
Definition
trointestinal tract, skin, genitourinary tract, thyroid
Multiple myeloma is a malignant clonal proliferation of
and central nervous system) and B symptoms. On ex-
plasma cells.
amination there is bulky lymphadenopathy and hep-
atosplenomegaly.
Incidence
Mycosis fungoides (S´ ezary’s Syndrome) is a rare cu-

3per 100,000.
taneous T cell lymphoma. The cells are trophic to
the skin particularly the hands and feet, and result
Age
in plaques and lumps of associated with generalised
Most commonly diagnosed 60–65 years.
lymphadenopathy.
Extranodal lymphomas can occur at any site of lym-

Sex
phoid tissue (gastrointestinal tract, salivary glands,
2M:1F
lung, thyroid, skin, gonads, bone and brain). Gas-
trointestinallymphomaisparticularlycommoninthe
Pathophysiology
MiddleEastandisalsoseeninassociationwithcoeliac
There is expansion of a single clone of plasma cells that
disease.
replace normal bone marrow and produce monoclonal
The staging of NHL is a measure of its extent and dis-
immunoglobulins. Cleavage of these immunoglobulins
tribution according to the Ann Arbor system, which
result in the production of Fab and Fc fragments; the Fab
is suffixed by B if B symptoms are present (see
fragment is termed the Bence-Jones protein and is found
Table 12.8)
in the urine of patients with myeloma. If the antibody
polymerises there may be hyperviscosity of the blood.
Investigations There is also production of osteoclast stimulation fac-
Thediagnosisismadebylymphnodebiopsy,cytogenetic tor causing lytic bone lesions, bone pain and hypercal-
studies of lymphoma cells may give prognostic informa- caemia. Incomplete immunoglobulins may precipitate
tion. Full blood count may show anaemia and throm- in the tissues as amyloid. Renal damage may result from
bocytopenia suggestive of marrow involvement. Staging deposition of light chains, amyloid or hypercalcaemia.