73-year-old female with a background of rheumatoid arthritis has been admitted to your ICU with a history of acute shortness of breath and a fluctuating conscious state. She has been having severe headaches for the last three weeks.
The following investigations were obtained:
Parameter |
Patient Value |
Normal Adult Ran e |
|
Haemoglobin |
76 |
1 15 - 160 |
|
White Cell Count |
23.8 x 10 |
4.0 - 1 1.0 |
|
Platelets |
198 x 10 IL |
150 - 450 |
|
Blood Film |
2% Plasma cells and rolueaux formation |
Parameter |
Patient Value |
Normal Adult Range |
Sodium |
137 mmol/L |
135 - 145 |
Potassium |
4.3 mmol/L |
3.2 -4.5 |
Chloride |
106 mmol/L |
100 - 1 10 |
Bicarbonate |
25 mmol/L |
22 - 27 |
Urea |
15.0 mmol/L* |
3.0 - 8.0 |
Creatinine |
280 umol/L* |
70 - 120 |
Total Calcium |
2.75 mmol/L* |
2.15 - 2.60 |
Phosphate |
1.3 mmol/L |
0.7 — 1.4 |
Albumin |
26 g/L* |
33 — 47 |
Globulins |
92.3 g/L* |
25 — 45 |
a) Give the diagnosis. (20% marks)
b) What urgent treatment is indicated? (10% marks)
a)
b)
a)
This looks like a hyperviscosity syndrome due to hypergammaglobulinaemia, a complication of Waldenström's macroglobulinemia or (less likely) multiple myeloma or plasmacytoma.
Features of this presentation which suggest hyperviscosity:
Other clinical features of symptomatic hyperviscosity include:
Even though multiple myeloma is quoted by the college, Waldenström's macroglobulinemia is the disorder where clinically significant hyperviscosity occurs most frequently, according to this article by Mehta et al (2003). This is because Waldenström's macroglobulinemia is characterised by the excess production of IgM, which is a massive molecule. However, numerous other causes of plasmacytosis exist:
b) Plasmapheresis is the usual urgent treatment for this. You centrifuge away the monoclonal badness and replace with nice clean albumin.
Mehta, Jayesh, and Seema Singhal. "Hyperviscosity syndrome in plasma cell dyscrasias." Seminars in thrombosis and hemostasis. Vol. 29. No. 05. Copyright© 2003 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.:+ 1 (212) 584-4662, 2003.