This paper is only available as a PDF. To read, Please Download here.
Abstract
Factor VIII: C inhibitors associated with primary amyloidosis have not been previously
reported. A patient with autopsy-proved primary amyloidosis developed renal failure
requiring peritoneal dialysis. Purpura and a prolonged partial thromboplastin time
(PTT) were first observed 3 years later, after treatment was changed from peritoneal
dialysis to hemodialysis. Plasma contained a time-dependent factor VIII:C inhibitor.
The inhibitor on isoelectric focusing showed two peaks of activity, one with an isoelectric
point (pl) of approximately 4 and the second larger, with a pi of approximately 8.
Both were neutralized only by antisera to IgA and κ light chain. A monoclonal antibody
prepared in Balb/c mice against the variable region of the κ light chain also blocked
the inhibitor. The delayed onset of the coagulopathy could be explained by the change
from peritoneal to hemodialysis, because in the former, significant amounts of the
paraprotein, indicated by an “M-spike,” were recovered in the dialysate. N-terminal
amino acid sequencing of the first 20 amino acids of the variable region of the kappa
light chain from the urinary protein and the splenic amyloid subunit showed identity.
Abbreviations:
EDTA (ethylenediaminetetraacetic acid), factor VIIIC procoagulant (glycoprotein absent in plasma of patients with severe hemophilia), factor VIIIC inhibitor (inhibitor directed against coagulant portion of factor VIII molecule), IgA (immunoglobulin A), IgG (immunoglobulin G), IgM (immunoglobulin M), pl (isoelectric point), PT (prothrombin time), PTT (partial thromboplastin time), SDS-PAGE (sodium dodecyl sulfate-polyacrylamide gel electrophoresis)To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Translational ResearchAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Acquired factor X deficiency in a patient with amyloidosis.Thromb Diath Haemorrh. 1962; 7: 558-566
- Deficit acquis en facteur X chez un malad atteint d'amylose primitive.Nouv Rev Fr Hematol. 1962; 2: 868-887
- Amyloidosis and factor X deficiency.South Med J. 1982; 75: 306-310
- Coagulopathy in amyloidosis: combined deficiency of factors IX and X.Am J Hematol. 1977; 3: 225-235
- Hemorrhagic diseases.in: Lee & Febiger, Philadelphia1975: 379
- Laboratory evaluation of hemostasis.in: Lea & Febiger, Philadelphia1967: 92
- Changes in maternal coagulation factors after intra-amniotic injection of hypertonic saline.Obstet Gynecol. 1971; 37: 660-666
- Laboratory methods in hematology and hemostasis.in: Pittiglio DH Sacher RA Clinical hematology and fundamentals of hemostasis. FA Davis, Philadelphia1987: 470-471
- A more uniform measurement of factor VIII inhibitors.Thromb Diath Haemorrh. 1975; 34: 869-871
- Agarose gel method: its usefulness in assaying factor VIII inhibitors, evaluating treatment and suggesting a mechanism of action for factor IX concentrates.Br J Haematol. 1985; 60: 739-750
- Inactivation of factor VIII coagulant activity by two different types of antibodies.Blood. 1982; 60: 1103-1109
- Determination of the von Willebrand's disease antigen (factor VIII-related antigen) in plasma by quantitative immunoelectrophoresis.J Lab Clin Med. 1975; 86: 152-159
- Procedures for the coagulation laboratory.in: Educational Products Division, American Society for Clinical Pathology, Chicago1981: 42-46
- A modified SDS agarose gel method for determining factor VIII von Willebrand factor multimers using commercially available reagents.Thromb Res. 1985; 39: 777-780
- An acquired inhibitor to factor VIII:C in nonhemophiliac: twenty years of observation and characterization.Am J Hematol. 1987; 24: 415-424
- Immunochemical characterization of antibodies to factor VIII in hemophiliac and nonhemophiliac patients.J Lab Clin Med. 1981; 97: 791-800
- Western blotting: electrophoretic transfer of proteins from sodium dodecyl sulfate-polyacrylamide gels to unmodified nitrocellulose and radiographic detection with antibody and radioiodinated protein A.Anal Biochem. 1981; 112: 195-203
- A dot-immunobinding assay for monoclonal and other antibodies.Anal Biochem. 1982; 119: 142-147
- Polymorphism in a kappa I primary (AL) amyloid protein (BAN).Mol Immunol. 1986; 23: 73-78
- A chemical study of antiovalbumin.Biochem J. 1950; 46: 473-478
- Polyquarternary amines prevent peptide losses from sequenators.Anal Biochem. 1978; 84: 622-627
- Rapid analysis of amino acid phenylthiohydantoins by higher performance liquid chromatography.Anal Biochem. 1977; 77: 569-573
- Regeneration of amino acids from thiazolamines formed in the Edman degradation.Anal Biochem. 1975; 68: 47-53
- A circulating anticoagulant in γ1A multiple myeloma: its modification by penicillin.J Clin Invest. 1965; 44: 1866-1881
- Immunology. An illustrated outline.in: CV Mosby, St. Louis1986: 24
- Acquired inhibitors against factor VIII and other clotting proteins.in: Colman RW Hirsh J Marder VJ Salzman EW Hemostasis and thrombosis: basic principles and clinical practice. JB Lippincott, Philadelphia1987: 825-840
- Heterogeneity of factor VIII antibodies: further immunochemical and biologic studies.Blood. 1977; 49: 807-817
- Acquired inhibitor to human factor VIII associated with paraproteinemia and subsequent development of chronic lymphatic leukemia.Br Med J. 1982; 285: 174-175
- Hemophilia-like disease associated with pregnancy.Obstet Gynecol. 1972; 40: 56-64
- Circulating anticoagulant in a macroglobulin with factor VIII inhibitory activity [Abstract].Ann Intern Med. 1968; 68: 1170
- A macroglobulin with inhibitory activity against coagulation factor VIII.Blood. 1970; 35: 370-376
- A hemorrhagic syndrome in Waldenström's macroglobulinemia secondary to immunoadsorption of factor VIII. Recovery after splenectomy.N Engl J Med. 1979; 300: 408-410
- Acquired factor X and antithrombin III deficiency in a patient with primary amyloidosis and nephrotic syndrome.Scand J Haematol. 1985; 35: 155-157
- Acquired factor X deficiency, altered plasma antithrombin activity and association with amyloidosis.JAMA. 1974; 230: 1658-1660
- Factor X deficiency in amyloidosis: a critical review.Am J Hematol. 1981; 11: 443-450
- Production and characterization of pepsin fragments of human IgA to determine domain specificity of monoclonal anti IgA antibodies.Immunol. 1986; 59: 153-158
- Heterogeneity of binding of human IgA subclasses to protein A.J Immunol. 1979; 123: 1457-1461
- Isoelectric focusing of human IgA and secretory proteins using thin layer agarose gels and nitrocellulose capillary blotting.J Immunol Methods. 1984; 66: 313-321
- Chronic peritoneal dialysis in a patient with primary amyloidosis, renal failure and factor X deficiency.South Med J. 1978; 71: 764-777
- Immunoglobulin and dextran losses during peritoneal dialysis.Arch Intern Med. 1974; 134: 266-269
- Protein loss in peritoneal dialysis.Lancet. 1964; 1: 738-741
- Identity of a peritoneal fluid immunoglobulin light chain and the amyloid fibril in primary amyloidosis.Arthritis Rheum. 1976; 19: 755-759
Article info
Publication history
Accepted:
November 8,
1988
Received in revised form:
November 1,
1988
Received:
June 23,
1988
Identification
Copyright
© 1989 Published by Elsevier Inc.
