History
David is a 72 year old retired teacher who lives with his 68 year old wife, Susan, in the Scottish Highlands. Their three adult children do not live nearby, but visit their parents when they can.
David has long-standing type 2 diabetes, managed with oral hypoglycaemics plus insulin. He also has long-standing moderate chronic kidney disease (CKD) and mild diabetic neuropathy. Susan has Parkinson’s disease; although she can carry out most activities of daily living (ADL), David does most of the housework and is her main carer.
David has seen his GP and received treatment for two chest infections in the past three months. His HbA1C levels indicate poor diabetes control and at a recent diabetic clinic review a blood test revealed a decrease in David’s kidney function from an eGFR of 42 ml/min (stage 3 CKD), six-months previously to 28 ml/min. David was referred to a nephrologist because his GP was concerned with the decline in his kidney function.
The nephrologist carried out an urgent assessment of possible causes of declining kidney function. Blood tests and serum and urinary protein electrophoresis and serum free light chain assays were ordered.
Blood tests showed:
- Haemoglobin level of 110 g/L (range 130 – 170 g/l)
- White cell count 2.8 x 109/L (range 4 – 11 x 109/l)
- Neutrophil count 0.7 x 109/L (range 2.5 – 7.5 x 109/l)
- Platelet count 200 x 109/L (range 150 – 400 x 109/l)
- Calcium 2.8 mmol/L (range 2.2 – 2.6 mmol/l)
- Urea 4.8 mmol/L (range 2.5 – 7.5 mmol/l)
- Creatinine 225 umol/L (range 70 – 150 umol/l)
- Total protein 74 g/L (60 – 80 g/l)
- Albumin 32 g/L (range 35 – 50 g/l)
- Serum b2 microglobulin was 5.2mg/L (range 0.3 – 1.9mg/l)
The test results showed no evidence of an intact immunoglobulin paraprotein, but a markedly elevated serum immunoglobulin light chain isotype, with a kappa FLC (κ FLC) of 7,200 mg/l; the lambda FLC (λ FLC) was 4.9 mg/l and κ/λ ratio 1,469. The results were suggestive of light chain only myeloma and David was therefore referred to a haematologist for further investigations.
A bone marrow biopsy showed infiltration by 78% kappa restricted plasma cells. The abnormal plasma cells were positive by CD56 and cyclin D1+ immunohistochemical staining. FISH analysis revealed no high-risk cytogenetic abnormalities and LDH was within normal limits. A skeletal survey was negative and AL amyloidosis was ruled out.
According to the proposed Revised International Staging System (R-ISS) David was classified as having Stage II myeloma [1].