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Case history 1

image A 68-year-old man complained of a few weeks’ history of worsening headaches and blurred vision. He also mentioned that his concentration had been impaired lately, especially when trying to read for any period of time. He had become increasingly breathless on exertion. A plain chest radiograph showed no obvious abnormality and a CT scan of his brain was unremarkable. Biochemistry revealed total protein 98 g/l, globulin 60 g/l and plasma viscosity 7.5 mPa·s. His full blood count showed a normocytic anaemia only, with a haemoglobin level of 95 g/l. Further investigation revealed the presence of an immunoglobulin M (IgM) paraprotein measuring 43 g/l.

What are the main symptoms of hyperviscosity syndrome (HVS)?

What abnormal findings may be present on clinical examination?

What are the main causes of HVS?

Which investigations would be useful?

How should this patient be treated?

What are the main symptoms of HVS?

HVS is characterized by increased blood viscosity, which may lead to visual and neurological disturbances (as experienced by this patient), mucocutaneous bleeding, fatigue, constitutional symptoms and occasionally symptoms related to congestive heart failure or ischaemia.1 The neurological symptoms are:

  • vertigo;

  • seizures;

  • headaches;

  • ataxia;

  • worsening somnolence and coma;

  • cognitive changes;

  • hearing loss;

  • stroke.

What abnormal findings may be present on clinical examination?

Clinical examination should include full cardiovascular (to look for evidence of heart failure or ischaemia) and neurological examinations, including fundoscopy (which may reveal venous engorgement, haemorrhages, exudates, microaneurysms and papilloedema).1 The presence or absence of bruising, epistaxis or gingival bleeding should also be established.

What are the main causes of HVS?

Increased viscosity usually results from an increase in plasma proteins (often serum immunoglobulins), as was the case in this patient. There is a variety of possible underlying causes:

  • plasma cell myeloma;

  • paraprotein-producing lymphoproliferative disorder (e.g. Waldenström’s macroglobulinaemia);

  • inflammatory disorder (e.g. rheumatoid arthritis, immunoglobulin G [IgG]-4-related disease);

  • cryoglobulinaemia.

The patient was likely to be suffering from Waldenström’s macroglobulinaemia. This disorder can lead to a decrease in blood flow in vital organs, causing symptoms of ischaemia in a whole range of tissues, especially in the cerebral microvasculature. The extra strain on the myocardium of pumping this viscous blood can sometimes lead to congestive heart failure.

Which investigations would be useful?

Investigations should include a full blood count, including a blood film, which may show red blood cell rouleaux. Biochemical analysis of plasma viscosity (if available), urea and electrolytes, uric acid and lactate dehydrogenase should be done. Total protein and serum globulin (which are included in liver function tests) should be measured and immunoglobulin and protein electrophoresis carried out. A clotting screen including fibrinogen may also be useful if haemorrhage is a presenting feature. If there is evidence of heart failure, an ECG, transthoracic echocardiogram and ...

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