Case Studies

 Case 6.9 Waldenströms macroglobulinaemia

A 76-year-old woman presented with a 6-month history of weakness, malaise, exertional dyspnoea and abdominal discomfort. In the previous month she had experienced two epistaxes but did not have headaches, visual disturbances, weight loss, bone pain or recurrent infections. On examination she was pale, with moderate axillary and cervical lymphadenopathy. Her liver and spleen were enlarged by 5cm.

On investigation, she had an ESR of 112mm/h and a haemoglobin of 108g/l. Her white-cell count and differential were normal. The total serum protein was increased to 130g/l. Protein electrophoresis and immunoelectrophoresis showed a dense paraprotein in the gamma region which proved to be an IgM of kappa type. Quantitation of the serum immunoglobulins showed normal IgG (9.4g/l) and IgA levels (1.1g/l), but her IgM was markedly raised at 64.5g/l (NR 0.5-2.0g/l). By densitometry (Chapter 19), the paraprotein measured 63g/l. Electrophoresis of concentrated urine showed no free monoclonal light chains and there were no bone lesions on X-rays of her chest and skull. Her serum viscosity, relative to water, was 4.7 (NR 1.4-1.8). A bone marrow examination showed a pleomorphic cellular infiltrate composed of a mixture of small lymphocytes, plasma cells and cells of an intermediate appearance, called lymphoplasmacytoid cells. These are features of Waldenström's macroglobulinaemia.