Essential monoclonal cryoglobulinaemia: the use of intermittent plasmapheresis to control cold induced symptoms*

Abstract

A case of essential monoclonal cryoglobulinaemia is described. Acute cold induced symptoms were rapidly controlled by repeated plasmapheresis. Oral cyclophosphamide chemotherapy was then able to maintain the IgG paraprotein at a level at which symptoms did not appear.

Cryoglobulinaemia and angiomatosis*

“…Management is symptomatic and measures should obviously be taken to avoid the cold. Plasmapheresis may be beneficial in the acute situation (James & Kingston, 1979). Our patient has remained reasonably well, but if she should relapse this would be the treatment of choice.…”

“…They are, however, completely benign. Masson (1923) was the first to describe a bizarre endothelial proliferation associated with organizing thrombi, which he called 'vegetant intravascular haemangioendothelioma'. In 1932, Henschen interpreted this endothelial proliferation as a reactive process rather than a neoplasm.…”

“…The process of thrombus organization has also been called 'intravascular angiomatosis' (Salyer & Salyer, 1975). The key microscopic feature is the presence of a capillary growth of hyperplastic endothelial cells supported by delicate fibrous stalks, entirely confined within the vascular lumen (Rosai & Akerman, 1974;Kuo, Sayers & Rosai, 1976;Clearkin & Enzinger, 1976). The lesions seen in cryoglobulinaemia are very similar and occur as a result of vessel blockage by cryoprecipitate with subsequent organization.…”

Cryoglobulinaemia mimicking dermatitis artefacta

“…Our patient appears to have 'essential' monoclonal cryoglobulinaemia in as much as there is no evidence of myeloma or other lymphoproliferative disease at present; Brouet et al (1974) found that about 20",, of patients with type I cryoglobulinaemia had no underlying pathology. James & Kingston (1979) have pointed out that there is no clear picture ofthe prognosis for these patients, but consider that some occult lymphoid or plasma cell neoplasm may become apparent in the future.…”

“…Plasmapheresis has been advocated as effective therapy for symptomatic relief in type I cryoglobulinaemia (Grey & Kohlcr, 1973), and similarly the use of cyclophosphamide as maintenance therapy has been reported as successful (Bengtsson et al, 1975). James & Kingston (1979) reported a patient with type I cryoglobulinaemia in whom cyclophosphamide was used as long-term therapy, saving plasmapheresis for beneficial relief of acute cold induced symptomatic flares of activity. Our patient has been advised about avoiding cold exposure, but as she intends to emigrate to a wanner climate the exposure is likely to be minimal.…”