Case 69 – part 3 – summary

During this short case we looked at serum protein electrophoresis.

case-69-part-3

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
  • Neuropathy
  • Cold aggultinins/haemolysis
  • Cryoglobulinaemia
  • Hyperviscosity – fundoscopy is vital to look for retinal haemorrhage
  • Infections

 

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

 

References

http://onlinelibrary.wiley.com/doi/10.1111/bjh.12760/pdf

 

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