A 71 year old lady with a history of malaise and weight loss is admitted to hospital overnight under a general medical team for investigations of blurry vision. The intensive care team was involved to offer advice when on the following morning some abnormal blood results became available.
|
Parameter |
Patient Value |
Normal Adult Range |
|
|
Haemoglobin |
76 |
115 - 160 |
|
|
White Cell Count |
23.8 x 10 |
4.0 - 1 1.0 |
|
|
Platelets |
198 x 10 / L |
150 - 450 |
|
|
Blood Film |
2% Plasma cells and rouleaux 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 |
The original college viva was:
Hematology OSCE
Data sets provided for interpretation included
a) Leukemoid reaction
b) A raised APTT, INR and low fibrinogen
c) A macrocytosis with an underlying myelodysplastic syndrome
d) A raised APTT which corrected with normal plasma
This was not a satisfying or informative OSCE stem, the viva was rewritten completely and is therefore not representative of what the college examiners would have wanted. The data set comes from Question 7.3, from the first paper of 2016.
Anyway: the major abnormalities are:
Overall, this sounds like multiple myeloma.
This is a form of reversible RBC aggregation, and it seems to be a cause of some altered blood rheology. The rouleaux are stacks of red blood cells which form due to macromolecule bridging between their surface molecules. In 1926 Eric Ponder published on the subject, and his paper contains beautifully drawn diagrams of his experimental design. In short, anything which might increase your ESR will cause rouleaux formation, and thus the differentials include a broad range of conditions:
This, in combination with the history and blood findings, suggests hyperviscosity. Specifically, dilated retinal veins suggests central retinal vein occlusion.
The major issue here is the hyperviscosity of the blood. Specific management of the myeloma would not result in a sufficiently rapid clearance of the paraprotein, and therefore a blood purification therapy such as plasmapheresis would be required.
Characteristics of a disease process which make plasmapheresis an effective option:
Undesirable blood components:
Desirable blood components which you'd rather keep:
Urgent plasma exchange:
Less urgent plasma exchange:
One should note that in their list of indications, the college noted some Grade II, III and IV recommendations, such as:
Disclaimer: the viva stem above may be an original CICM stem, acquired from their publicly available past papers. Or, perhaps it is a slightly altered version of the original CICM stem. Or, it is a completely original viva stem, concocted by the monstrously amoral author of Deranged Physiology for nothing more than his own personal amusement. In either case, because the college do not make the main viva text or marking criteria available, almost everything here has been confabulated. It might sound like a plausible viva and it could be used for the purpose of practice, but all should be aware that it does not represent the "true" canonical CICM viva station.
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.
Triger, D. R., and Ralph Wright. "Hyperglobulinaemia in liver disease." The Lancet 301.7818 (1973): 1494-1496.
McLeod, Bruce C. "Therapeutic apheresis: use of human serum albumin, fresh frozen plasma and cryosupernatant plasma in therapeutic plasma exchange."Best Practice & Research Clinical Haematology 19.1 (2006): 157-167.
Reimann, P. M., and P. D. Mason. "Plasmapheresis: technique and complications." Intensive care medicine 16.1 (1990): 3-10.
Winters, Jeffrey L. "Plasma exchange: concepts, mechanisms, and an overview of the American Society for Apheresis guidelines." ASH Education Program Book 2012.1 (2012): 7-12.
Oh's Manual: Chapter 97 (pp. 993) Therapeutic plasma exchange and intravenous immunoglobulin therapy by Ian Kerridge, David Collins and James P Isbister.
Szczepiorkowski, Zbigniew M., et al. "Guidelines on the use of therapeutic apheresis in clinical practice—Evidence‐based approach from the apheresis applications committee of the American Society for Apheresis." Journal of clinical apheresis 25.3 (2010): 83-177.
Russi, Gianpaolo, and Piero Marson. "Urgent plasma exchange: how, where and when." Blood Transfusion 9.4 (2011): 356.