The half-life of immune globulin G in chronic benign cryptogenic hypergammaglobulinaemic purpura with pigmentation. And a report of a case.

an entity, to record the high level of immune globulin G, and to report that the half-life of the immune globulin G in this case is normal or diminished. The elevation of the serum immune globulin G is due to excessive production, and not to slow or diminished removal from the plasma. Waldenstrom reported the syndrome in 1943, and then reported this and a different dysproteinaemic disease, macroglobulinaemia, in a single paper in 1948. Physicians are still sometimes confused because the hyperg}obulinaemia and the macroglobulinaemia both have borne his name. It has also been confusing that purpura occurs in the course of several hyperglobulinaemic diseases. Chronic benign cryptogenic hypergammaglobulinaemia with purpura and skin pigmentation differs from macroglobulinaemia inter alia in the patient remaining generally well, the course being benign, the bone marrow being normal, the lymph glands not being enlarged, and the dysproteinaemia being an excess of immune globulin G and not an excess of a macroglobulin. It is more rare than macroglobulinaemia. Not every case of purpura and hyperglobulinaemia is Waldenstrom's hyperglobulinaemic purpura, which is very uncommon. It is characterised by frequent crops of small purpuric spots, repeated over many years, predominantly on the lower legs, the spots being obscured in time by permanent heavy brown pigmentation of the affected skin. The serum protein electrophoresis shows a high peak in the gammaglobulin area. The main rise is in the immune globulin G fraction which reaches 3 grammes and over. There may be occasional swelling of a parotid gland. The lacrimal glands may be affected, with some diminution of tear production. Corneal damage may occur and probably is in some degree due to dryness of the eyes. Dryness of the mouth is not severe, nor is the dryness of the eyes. As time goes by, the pigmentation of the lower legs deepens. Eventually the pigmentation is much more striking than the original purpura, and then, though the purpura still occurs, it can hardly be seen. Small keratoses may appear in the pigmented skin. The disease lasts so long as life itself. The only serious disability is impairment of sight if the corneal damage is severe. There is no bleeding tendency. The cause is unknown. The dark skin and the protein changes are reminiscent of kala-azar, but no infection or infestation has been identified.