During this short case we looked at serum protein electrophoresis.
This showed a band in the gamma region and immunofixation confirmed a IgM kappa paraprotein. IgM paraproteins are commonly associated with MGUS, low grade lymphoma and rarely myeloma. Typically IgM paraproteins are associated with lymphoplasmacytic lymphoma/Waldrenstrom’s macroglobulinaemia or splenic marginal zone lymphoma.
When evaluating a person with a paraprotein it is important to think of the following:
- Lymphoma – lymphadenopathy, splenomegaly, B symptoms
- Myeloma – bone pain, symptoms of hypercalcaemia
- Renal failure – amyloidosis, tumour lysis, light chain deposition
- Cold aggultinins/haemolysis
- Hyperviscosity – fundoscopy is vital to look for retinal haemorrhage
It would be important to consider the following investigations:
- Biochemistry: LDH, U&E, LFT, calcium
- Microbiology: HIV, hep B and hep C
- Bone marrow aspirate and trephine biopsy (looking for a lymphoplasmacytic infiltrate with a null B cell phenotype)
- Molecular analysis for MYD88 and possibly CXCR4
- CT chest/abdomen/pelvis
This patient has hyperviscosity so treatment with steroids e.g. dexamethasone 20mg and plasma exchange is indicated. Do not give rituxumab until the IgM paraprotein has settled as there is a risk of IgM flare. Common regimes include dexamethasone, cyclophosphamide rituxumab or bendamustine ritxuimab
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