Question 7.3

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)

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College Answer

Hyperviscosity syndrome secondary to multiple myeloma.

Plasmapheresis.

Discussion

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:

Acute shortness of breath, which suggests heart failure
A fluctuating conscious state, which suggests cerebrovascular disease
Severe headaches, which suggests cerebrovascular disease
Leukocytosis, which suggests haematological malignancy
An elevated globulin level
An elevated plasma cell count, which suggests plasmacytoma or Waldenström's macroglobulinemia

Other clinical features of symptomatic hyperviscosity include:

Constitutional symptoms: fatigue, malaise, and shortness of breath.
Haemorrhagic symptoms: gingival or mucosal bleeding, or epistaxis.
Blurred vision due to central retinal vein occlusion
Headaches due to increased intracranial pressure (due to venous occlusion)
Fundoscopy reveals dilated, tortuous retinal veins, as well as flame-shaped haemorrhages.
Stroke, seizures, coma (usually, venous cerebral infarction)
Renal failure
Aggravated heart failure (more difficult to pump the viscous blood)
Priapism

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:

MGUS
Multiple myeloma
Waldenström's macroglobulinemia
Solitary plasmacytoma
Systemic AL amyloidosis
POEMS syndrome
Adenocarcinoma of the colon
Pulmonary tuberculosis
Cirrhosis of the liver
Aplastic anaemia
Syphilis
Leprosy
Varicella zoster infection
Serum sickness
Primary infection and reactivation of Epstein–Barr virus
Acute respiratory infections
Parvovirus B19 infection
Rubella
Hepatitis virus A infection

b) Plasmapheresis is the usual urgent treatment for this. You centrifuge away the monoclonal badness and replace with nice clean albumin.

References

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.

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