A 79-year-old woman presents with mild dyspnoea and confusion. Of note in her past medical history was a one year history of Raynaud's phenomenon.
On examination her pulse was 118 beats per minute, she had a blood pressure of 122/88 mmHg and she had a small ulcer on her right big toe.
Auscultation of her chest revealed bibasal crackles and she had mild ankle oedema.
Her investigations show:
Haemoglobin: 9.5 g/dl (11.5-16.5)
White cell count: 3.5 x 109/l (4-11)
Platelet count: 110 x 109/l (150-400)
Serum total protein: 120 g/l (61-76)
Serum immunoglobulins:
IgA: 0.8 g/l (0.8-3.0)
IgG: 15 g/l (6.0-13.0)
IgM: 70 g/l (0.4-2.5)
Which of the following complications is she likely to develop?
(Please select 1 option)
0. Acute renal failure
0. Atypical pneumonia
o. Erythema repens gyratum
o. Hyperviscosity syndrome
o. Pathological bone fracture
Answer:Hyperviscosity syndrome
This elderly woman has a very raised IgM level, pancytopaenia, Raynaud's phenomenon and a foot ulcer.
The most likely diagnosis here is Waldenström's macroglobulinaemia (WM). WM refers to a condition that presents in the seventh or eighth decade of life.
It is characterised by the presence of a high level of a macroglobulin (immunoglobulin M [IgM]), elevated serum viscosity and the presence of a lymphoplasmacytic infiltrate in the bone marrow, resulting in pancytopaenias.
Raynaud's phenomenon may herald the onset of this condition and is due to cryoglobulinaemia.
The monoclonal IgM causes
hyperviscosity syndrome
cryoglobulinaemia types 1 and 2
coagulation abnormalities
polyneuropathies
cold agglutinin disease and anaemia
primary amyloidosis
tissue deposition of amorphous IgM in skin, the GI tract, kidneys, and other organs.