Repeated plasma exchanges were very useful for the treatment of a case of primary macroglobulinemia complicated with intraventricular hemorrhage.
A 43-year-old woman was diagnosed as primary macroglobulinemia, because of hemorrhagic diathesis, hepatosplenomegaly, high concentrations of monoclonal serum IgM-κ, characteristic ocular fundus and bone marrow infiltration in June, 1981. In January, 1983, the patient suddenly developed severe headache, nausea and vomiting. CT scan showed intraventricular hemorrhage. The patient's serum IgM level was rapidly decreased from 5,628 mg/dl to 683 mg/dl after three plasma exchanges performed at 24 hour intervals. Plasma exchange on a regular basis was started in February, 1983, and two plasma exchanges were performed at 24 hour intervals every 4th week.
Plasma exchanges were performed by continuous blood-cell separater using CS3000. Plasma was replaced by an equal volume of 3% dextran 40 in RL-8. Serial studies revealed that immediately after the exchange transfusion there was significant prolongation of the prothrombin time and the activated partial thromboplastin time with reduction of the fibrinogen, antithrombin-III, plasminogen and α₂-macroglobulin. Platelet counts revealed significant decrease. All hemostatic parameters had returned to almost normal levels by 24 hours after the cxchange. The clinical hemorrhagic episodes did not develop despite abnormal coagulation parameters.
The 3% dextran 40-electrolyte solution is useful fot the replacement solution in the plasma exchange therapy of primary macroglobulinemia.