Summary
Three patients with a history of chronic idiopathic thrombocytopenic purpura stretching back over 20 years are reported. Despite splenectomy and immunosuppressive therapy satisfactory control of their disease has not been achieved. They had remained refractory to all therapeutic manoeuvres with corticosteroids and immunosuppressives for years with thrombocyte counts between 5,000 and 25,000/μl and the concommitant risk of bleeding.
This report describes the treatment of bleeding complications in these patients with high dose intravenous immunoglobulin; the peripheral blood thrombocyte count increased in all three patients from subnormal towards normal, but 2 to 4 weeks later returned to its initial low value.
During the therapeutically induced raised thrombocyte count a normal bleeding time and only a moderate inhibition of thrombocyte adhesion and aggregation was observed resulting in reasonable haemostasis. High dose intravenous immunoglobulin is therefore a practical method for the control of bleeding complications in patients with refractory chronic idiopathic thrombocytopenic purpura. A clear explanation for its mode of action has not been found — the lymphocyte subpopulations remained unchanged and immunoglobulin production in vitro during the course of treatment was only minimally decreased.
Similar content being viewed by others
References
Ahn YS, Byrnes JJ, Harrington WJ, Cyer ML, Smith DS, Brunskill DE, Pall LM (1978) The treatment of idiopathic thrombocytopenia with vinblastine-loaded platelets. N Engl J Med 298: 1101–1107
Born GVR (1962) Aggregation of blood platelets by adenosine diphosphate and its reversal. Nature 194: 927–929
Burger T, Schmelczer K, Kett K, Kutas J (1978) Immune thrombocytopenic purpura (ITP): A diagnostic and therapeutic survey of 86 cases with regard to the results of splenectomy and conservative therapy. Acta Med Acad Sci Hung 35: 213
Bussel JB, Kimberly RP, Inman RD, Schulman I, Cunningham-Rundles C, Cheung N, Smithwick EM, O'Malley J, Barandun S, Hilgartner MW (1983) Intravenous gammaglobulin treatment of chronic idiopathic thrombocytopenic purpura. Blood 62: 480–486
Clancy R, Jenkins E, Firkin B (1972) Qualitative platelet abnormalities in idiopathic thrombocytopenic purpura. N Engl J Med 286: 622–626
Fehr J, Hofmann V, Kappeller U (1982) Transient reversal of thrombocytopenia in idiopathic thrombocytopenic purpura by high-dose intravenous gammaglobulin. N Engl J Med 306: 1254
Harrington WJ, Sprague EC, Minnich V, Moore, CV, Aulvin RC, Dubach R (1953) Immunologic mechanism in idiopathic and neonatal thrombocytopenic purpura. Ann Intern Med 38: 433
Hellem AJ (1970) Platelet adhesiveness in von Willebrand's disease: A study with a new modification of the glass bead filter method. Scand J Haematol 7: 374–382
Imbach P, d'Apuzzo V, Barandun S et al. (1981) High-dosis intravenous gammaglobulin for idiopathic thrombocytopenic purpura in childhood. Lancet 1: 1228
Jiji RM, Firozvi T, Spurling CL (1973) Chronic idiopathic thrombocytopenic purpura. Treatment with steroids and splenectomy. Arch Intern Med 132: 380
Kelly BS, Levy JG, Sikora L (1979) The use of the enzyme-linked immunosorbent assay (ELISA) for the detection and quantification of specific antibody from cell cultures. Immunology 37: 45
MacPherson AIS, Richmond J (1975) Planned splenectomy in treatment of idiopathic thrombocytopenic purpura. Br Med J 64: 5949
Mielke CH Jr, Kaneshiro MM, Maher IA, Weiner JM, Rapaport SI (1969) The standardized normal Ivy bleeding time and its prolongation by aspirin. Blood 34: 204–215
Mueller-Eckhardt C, Mahn I, Schulz G, Mueller-Eckhardt G (1978) Detection of platelet antibodies by a radioactive antiimunoglobulin test. Vox Sang 35: 357
Pawelski S, Konopka L, Zdiechowska H (1981) Recurrence of thrombocytopenia in patients splenectomized for idiopathic thrombocytopenic purpura. Blut 43: 355–360
Picozzi VJ, Roeske WR, Creger WP (1980) Fate of therapy failures in adult idiopathic thrombocytopenic purpura. Am J Med 69: 690
Reinherz EL, Penta AC, Hussey RE, Schlossman SF (1981) A rapid method for separating functionally intact human T lymphocytes with monoclonal antibodies. Clin Immunol Immunpathol 21: 257–266
Salama A, Mueller-Eckhardt C, Kiefel V (1983) Effect of intravenous immunoglobulin in immune thrombocytopenia. Competitive inhibition of reticuloendothelial system function by sequestration of autologous red blood cells? Lancet 2: 193
Schmidt RE, Budde U, Schafer G, Streohmann I (1981) High dose intravenous gammaglobulin for idiopathic thrombocytopenic purpura. Lancet 2: 475
Schwartz SI, Hoep LM, Sachs S (1980) Splenectomy for thrombocytopenia. Surgery 88: 497
Seifried E, Gaedicke G, Pindur G, Rasche H (1984) The treatment of haemophilia A inhibitor with high dose intravenous immunoglobulin. Blut 48: 397
Schulman NR, Marder VJ, Weinrach RS (1965) Similarities between known antiplatelet antibodies and the factor responsible for thrombocytopenia in idiopathic purpura: physiologic, serologic and isotopic studies. Ann NY Acad Sci 124: 499–542
Schulman NR, Weinrach RS, Libre EP, Andrews HL, Shannon JA (1965) The role of the reticuloendothelial system in the pathogenesis of idiopathic thrombocytopenic purpura. Trans Assoc Am Phys 78: 374–390
Slichter SJ, Schwartz K (1979) Mechanism of action of vinblastine-loaded platelets (plts) in the treatment of chronic idiopathic thrombocytopenic purpura (ITP). Clin Res 27: 307 A
Stuart MJ, Kelton JG, Allen JB (1981) Abnormal platelet function and arachidonate metabolism in chronic idiopathic thrombocytopenic purpura. Blood 58: 326–329
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Seifried, E., Pindur, G., Stötter, H. et al. Treatment of refractory chronic idiopathic thrombocytopenic purpura with high dose intravenous immunoglobulin. Blut 48, 369–376 (1984). https://doi.org/10.1007/BF00319965
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00319965