Myeloma Presenting with Massive Splenomegaly

Myeloma typically presents with skeletal damage, renal failure, metabolic disturbances and anaemia. Less usual associations are polyneuropathy, solitary plasmacytomas, and only rarely is lymphadenopathy found. Discomfort and left upper quadrant pain due to marked splenomegaly appears unknown, particularly as the initial symptom. Accordingly, two cases are reported in which this was the reason for seeking medical attention. In both splenectomy was unavoidable because of size and symptomatic infarction. The dominant histologic feature in the first was striking proliferation of sinus lining cells engorged with the IgA paraprotein. In the second there was dramatic expansion of the red pulp. In neither, despite using appropriate immunohistochemical techniques, could the enlargement of the spleen be attributed to infiltration by plasma cells that in the peripheral and blood and marrow had a distinctively unusual morphology. In these two individuals the pathophysiology remains obscure. In the first, although considered, it was not possible to meet the criteria for this being an example of littoral cell angioma. In the second, a postulated disseminated plasmacytoma with primary splenic sequestration remains unproven. This unique finding is, therefore, presented as descriptive pathology without recognisable explanation.