Circulating immune complexes pre and post clotting factor infusion in hemophilia
Gomperts, E.D.; Jordan, S.; Berg, D.; Sakai, R.; Boylen, L.
Thrombosis and Haemostasis 46(4): 694-698
1981
ISSN/ISBN: 0340-6245 PMID: 6800050 Document Number: 181626
Circulating immune complexes (CIC) were studied by the Raji cell radioimmunoassay (Raji-RIA) and C-1q [q fragment of complement component 1]-solid phase assay (C1q-SPA) in sera samples obtained before and after replacement infusion from 21 young hemophiliac patients (mean age 9.5 yr). Seven patients had repeat studies. Of the patients, 16 were selected randomly and the other 5 were selected as having potential infusion related disease processes. All patients had received multiple infusions prior to entering the study. Five patients were infused with FIX [factor IX] concentrates (2 hemophilia B, 1 FVII [factor VII] deficiency, 2 with inhibitors to FVIII [factor VIII]. Of the hemophilia A patients (13 severe; 3 mild/moderate), 6 were infused with ABO-Rh type specific cryoprecipitate and 10 were infused with factor concentrate. Preinfusion sera samples evaluated by the Raji-RIA showed that 14 of 28 (50%) were sera positive. Of these 28, 11 (39.3%) were positive post infusion in the Raji-RIA. Infusion studies of the same sera samples using the C1q-SPA showed 5 of 28 (17.9%) positive pre-infusion and 4 of 28 (14.3%) positive post-infusion. No statistically significant relationship between pre- and post-infusion studies could be demonstrated. There was no obvious correlation between CIC levels and patient age, race, type of infusate, ABO-Rh blood type, hepatosplenomegaly, the presence or absence of HBsAg (hepatitis B surface antigen), antibody or chronic joint changes. CIC were detected in the 3 patients with SGOT (serum glutamic oxalacetic transaminase)/SGPT (serum glutamic pyruvic transaminase) levels greater than 5 times normal, in both patients with chronic renal disease (chronic glomerulonephritis), and 2 children who had previously demonstrated a Coombs'-positive hemolytic anemia after factor concentrate infusion. Six of 8 patients with extremely high levels of CIC had associated clinical or laboratory abnormalities. CIC (as detected by the Raji-RIA) are evidently present in increased frequency in hemophiliac patients. Although individual patients developed CIC post infusion, there is no clinical or statical data supporting a common infusate immunogen. CIC were present in patients with serum hepatitis and other immune related disorders, probably secondary to factor infusion. The immunopathogenicity and origin of CIC in hemophiliacs in uncertain.