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)
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
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
cryoglobulinaemia types 1 and 2
cold agglutinin disease and anaemia
tissue deposition of amorphous IgM in skin, the GI tract, kidneys, and other organs.