Multiple plasma exchanges successfully maintain a young adult patient with Crigler-Najjar syndrome type I.

Plasmapheresis has been shown to reduce total and free bilirubin levels in acute exacerbations of Crigler-Najjar syndrome, type I (CNS-TI), but its effectiveness in long-term management has not been reported. An 18-year-old (yo) male with CNS-TI, who required prolonged daily high-intensity phototherapy to prevent cerebral nervous system symptoms, developed increasingly frequent bouts of confusion, nausea, and vomiting associated with free bilirubin concentrations (fbcs) greater than 10-15 nmol/L. Pending consideration of orthotopic liver transplantation, plasma exchange (approximately 3 liters per procedure) was begun in 12/84 using the IBM/COBE 2997 with 5% albumin as replacement fluid. Frequency of treatments was guided by twice weekly fbcs, with plasma exchange for fbc greater than 10-15 nmol/L. Pre-exchange and postexchange fbcs ranged from 27.5 to 11 nmol/L and 9.2 to 2 nmol/L, respectively. Seventy-two exchanges were performed over a 28 month period. Irreversible CNS damage did not occur, and the patient underwent successful liver transplantation in April of 1987, with complete correction of his metabolic disorder. He remains well 18 months following transplantation.

Frozen storage of 11 units of sickle cell red cells for autologous transfusion of a single patient.

A patient with sickle cell disease and multiple alloantibodies required frozen storage of autologous red cells (RBC) to provide the RBC volume equivalent of 6 units of normal donor blood for an orthopedic surgical procedure. Eleven 600-ml donations were made at 4-week intervals, without significant change in hemoglobin concentration, hematocrit, or reticulocyte count, and with only small decreases in serum iron and ferritin concentrations. Minor modifications of a glycerolization and deglycerolization procedure described recently were used. We report in vitro recoveries of deglycerolized RBC from the 11 units following frozen storage for 27 to 391 days. Despite use of a single donor and adherence to a standardized processing protocol, considerable variation in the percent in vitro RBC recovery was observed (51-81%). In vivo survival of an aliquot following frozen storage for 27 days was identical with fresh autologous RBC (100% at 1 hr, 88% at 24 hr, one-half disappearance of 7.5 days). Autologous transfusion of RBC recovered from 7 units after storage for as long as 126 days was uneventful.

In vivo instability of red-blood-cell-bound C3d and C4d.

Until now, there have been no measurements of the in vivo stability of red-blood-cell-bound C3d and C4d subfragments of the third and fourth components of human complement. We have recently described a radiolabeled antiantiglobulin method for measuring RBC-bound C3d and have demonstrated that small amounts of C3d are present on RBC of all normal subjects tested. In the present study, the method was applied to follow the increments above baseline of RBC-bound C3d and C4d produced by autotransfusing 3 normal volunteers with 160-200 ml of RBC strongly coated in vitro by C3d and C4d. Posttransfusion measurements were carried out over 21-34 days. Immediate and long-term in vivo survival of the transfused RBC was unimpaired by C3d and C4d coating. Of the bound C3d antigen, 85%-95% disappeared from circulating RBC in 5-8 days; the remainder disappeared more slowly, with half-times in the range of 8-29 days. C4d antigen disappeared substantially more slowly, describable by a single exponential function in 2 of the 3 subjects, with half-times in the range of 12-31 days. Recognition of the in vivo instability of RBC-bound C3d helps in interpreting steady-state and changing levels of RBC C3d coating in a variety of alloimmune and autoimmune disorders.