1. Table 21.1 Diseases associated with
monoclonal immunoglobulins.
Neoplastic
Multiple myeloma
Solitary plasmacytoma
Monoclonal gammopathy of undetermined
significance (MGUS)
Waldenström macroglobulinaemia
Non-Hodgkin lymphoma
Chronic lymphocytic leukaemia
Primary amyloidosis
Heavy-chain disease
Benign
Chronic cold haemagglutinin disease
Transient (e.g. with infections)
HIV infection
Gaucher’s disease
Table 21.2 Plasma cell neoplasms (WHO,
2008).
Monoclonal gammopathy of undetermined
significance (MGUS)
Plasma cell myeloma
Variants:
Asymptomatic (smouldering) myeloma
Non-secretory myeloma
Plasma cell leukaemia
Plasmacytoma
Solitary plasmacytoma of bone
Extraosseous (extramedullary) plasmacytoma
Immunoglobulin deposition diseases
Primary amyloidosis
Systemic light and heavy chain deposition
diseases
Osteosclerotic myeloma (POEMS syndrome)
Table 21.3 The chemical structure of immune modulator drugs.
NH2
O
N
O
O
O
N
Thalidomide
Side effects
Neuropathy
Constipation
Sedation
DVT
O
O
O
N
N H
Lenalidomide
Side effects
Myelosuppression
Skin rash
DVT
O
Side effects
Myelosuppression
Fatigue
Neuropathy
NH2
O
O
N
N H
Pomalidomide
O
DVT, deep vein thrombosis.

2. Table 21.4 Features of benign and malignant paraproteinaemia.
Benign Malignant
Bence-Jones proteinuria Absent May be present
Serum paraprotein concentration Usually <30 g/L and
stationary
Usually >30 g/L and rising
Serum free light chain ratio Normal Abnormal
Immuneparesis
Absent Present
(hypogammaglobulinaemia)
Underlying lymphoproliferative disease or
myeloma
Absent Present
Bone lesions Absent Present
Plasma cells in marrow <10% >10%
Table 21.5 Classification of amyloidosis: types, structure and organ involvement.
Type Chemical nature Organs involved
Systemic AL amyloidosis
Associated with myeloma, Waldenström’s
Immunoglobulin light
macroglobulinaemia or MGUS
chains (AL)
May also occur on its own as primary
amyloidosis (associated with an occult
plasma cell proliferation)
May also occur in localized form with local
‘immunocyte’ proliferation
Tongue
Skin
Heart
Nerves
Connective tissue
Kidneys
Liver
Spleen
Reactive systemic AA amyloidosis
Rheumatoid arthritis, tuberculosis,
bronchiectasis, chronic osteomyelitis,
inflammatory bowel disease, Hodgkin
lymphoma, carcinomas, familial
Mediterranean fever
Protein A (AA) Liver
Spleen
Kidneys
Bone marrow
Familial amyloidosis e.g. Transthyretin
abnormalities
Nerves
Heart
Eyes
Localized amyloidosis
Central nervous system
Endocrine
Senile
β-Amyloid protein
Peptic hormones
Various
Alzheimer’s disease
Endocrine tumours
Heart, brain, joints, prostate, etc.
AA, AL, these are defined by their chemical nature as in the table; MGUS, monoclonal gammopathy of undetermined
significance.