Summary
Platelet destruction by antibodies is a common cause, of thrombocytopenia. In this review, various treatments for immune thrombocytopenia are discussed, with emphasis on corticosteroids, splenectomy, danazol, high-dose immunoglobulin G, anti-Rhesus globulin, colchicine and cytotoxic, immuftosuppressive agents such as cyclophosphamide and azathioprine. An approach to the treatment of adult idiopathic thrombocytopenic purpura (ITP) is reviewed in detail and the treatments for several other immune thrombocytopenic disorders are summarised.
Similar content being viewed by others
References
Abrams DI, Kiprov DD, Goedert JJ, Saragadharan MG, Gallo RC, et al. Antibodies to human T-lymphotropic virus type III and development of the acquired immunodeficiency syndrome in homosexual men presenting with immune thrombocytopenia. Annals of Internal Medicine 104: 47–50, 1986
Adner MM, Fisch GR, Starobin SG, Aster RH. Use of ‘compatible’ platelet transfusions in treatment of congenital isoimmune thrombocytopenic purpura. New England Journal of Medicine 280: 244–247, 1969
Ahn YS, Harrington WJ, Seelman RC, Eytel CS. Vincristine therapy of idiopathic and secondary thrombocytopenias. New England Journal of Medicine 291: 376–380, 1974
Ahn YS, Harrington WJ, Simon SR, Mylvaganam R, Pall RM, et al. Danazol for the treatment of idiopathic thrombocytopenic purpura. New England Journal of Medicine 308: 1396–1399, 1983
Ahn YS, Mylvaganam R, Garcia RO, Kim CI, Palow D, et al. Low-dose danazol therapy in idiopathic thrombocytopenic purpura. Annals of Internal Medicine 107: 177–181, 1987
Ahn YS, Rocha R, Mylvaganam R, Garcia R, Duncan R, et al. Long-term danazol therapy in autoimmune thrombocytopenia: unmaintained remission and age-dependent response in women. Annals of Internal Medicine 111: 723–729, 1989
Akwari OE, Itani KM, Coleman RE, Rosse WF. Splenectomy for primary and recurrent immune thrombocytopenic purpura (ITP): current criteria for patient selection and results. Annals of Surgery 206: 529–541, 1987
Ambriz P, Munoz R, Quintanar E, Sigler L, Aviles A, et al. Accessory spleen compromising response to splenectomy for idiopathic thrombocytopenic purpura. Radiology 155: 793–796, 1985
Ambriz R, Munoz R, Pizzuto J, Quintanar E, Morales M, et al. Low-dose autologous in vitro opsonized erythrocyte: radioimmune method and autologous opsonized erythrocytes for refractory autoimmune thrombocytopenic purpura in adults. Archives of Internal Medicine 147: 105–108, 1987
Ambriz R, Pizzuto J, Morales M, Chavez G, Guillen C, et al. Therapeutic effect of danazol on metrorrhagia in patients with idiopathic thrombocytopenic purpura (ITP). Nouvelle Revue Française d’Hématologie 28: 275–279, 1986
Arrowsmith JB, Dreis M. Thrombocytopenia after treatment with danazol. Correspondence. New England Journal of Medicine 315: 585, 1986
Ballin A, Andrew M, Ling E, Perlman M, Blanchette V, et al. High-dose intravenous gammaglobulin therapy for neonatal autoimmune thrombocytopenia. Journal of Pediatrics 112: 789–792, 1988
Barton JC, Herrera GA, Galla JH, Bertoli LF, Work J, et al. Acute cryoglobulinemic renal failure after intravenous infusion of gamma globulin. American Journal of Medicine 82(3): 624–629, 1987
Baumann MA, Menitove JE, Aster RH, Anderson T. Urgent treatment of idiopathic thrombocytopenic purpura with single-dose gammaglobulin infusion followed by platelet transfusion. Annals of Internal Medicine 104: 808–809, 1986
Becker T, Kuenzlen E, Salama A, Mertens R, Kiefel V, et al. Treatment of childhood idiopathic thrombocytopenic purpura with Rhesus antibodies (anti-D). European Journal of Pediatrics 145: 166–169, 1986
Bellucci S, Bordessoule D, Coiffier B, Tabah I. Interferon alpha-2b therapy in adult chronic thrombocytopenic purpura (ITP). Correspondence. British Journal of Haematology 73: 578–579, 1989
Bellucci S, Charpak Y, Chastang C, Tobelem G, the Cooperative Group on Immune Thrombocytopenic Purpura. Low doses v conventional doses of corticoids in immune thrombocytopenic purpura (ITP): results of a randomized clinica trial in 160 children, 223 adults. Blood 71: 1165–1169, 1988
Boshkov LK, Kelton JG. Use of intravenous gammaglobulin as an immune replacement and an immune suppressant. Transfusion Medicine Review 3: 82–120, 1989
Boughton BJ, Chakraverty R, Baglin TP, Simpson A, Galvin G, et al. The treatment of chronic idiopathic thrombocytopenia with anti-D (Rho) immunoglobulin: its effectiveness, safety and mechanism of action. Clinical and Laboratory Haematology 10: 275–284, 1988
Brox AG, Howson-Jan K, Fauser AA. Treatment of idiopathic thrombocytopenic purpura with ascorbate. British Journal of Haematology 70: 341–344, 1988
Bussel JP, Berkowitz RL, McFarland JG, Lynch L, Chitkara U. Antenatal treatment of neonatal alloimmune thrombocytopenia. New England Journal of Medicine 319: 1374–1378, 1988a
Bussel JB, Pham LC, Aledort L, Nachman R. Maintenance treatment of adults with chronic refractory immune thrombocytopenic purpura using repeated intravenous infusions of gammaglobulin. Blood 72: 121–127, 1988b
Bussel JB, Saal S, Gordon B. Combined plasma exchange and intravenous gammaglobulin in the treatment of patients with refractory immune thrombocytopenic purpura. Transfusion 28: 38–41, 1988c
Caplan SN, Berkman EM. Immunosuppressive therapy of idiopathic thrombocytopenic purpura. Medical Clinics of North America 60: 971–986, 1976
Cattaneo M, Gringeri A, Capitanio AM, Santagostino E, Mannucci PM. Anti-D immunoglobulin for treatment of HIV-related immune thrombocytopenic purpura. Correspondence. Blood 73: 357, 1989
Chong BH, Ismail F, Cade J, Gallus AS, Gord S, et al. Heparininduced thrombocytopenia: studies with a new low molecular weight heparinoid, Org 10172. Blood 73: 1592–1596, 1989
Cimo PL, Aster RH. Post-transfusion purpura: successful treatment by exchange transfusion. New England Journal of Medicine 287: 290–292, 1972
Clarkson SB, Bussell JB, Kimberly RP, Valinsky JE, Nachman RL, et al. Treatment of refractory thrombocytopenic purpura with an anti-Fcτ-receptor antibody. New England Journal of Medicine 314: 1236–1239, 1986
Cola B, Tonielli E, Sacco S, Brulatti M, Franchini A. Surgical treatment of chronic idiopathic thrombocytopenic purpura: results in 107 cases. International Surgery 71: 195–198, 1986
Cole CW, Bormanis J. Ancrod: a practical alternative to heparin. Journal of Vascular Surgery 8: 59–63, 1988
Coon WW. Splenectomy for idiopathic thrombocytopenic purpura. Surgery, Gynecology and Obstetrics 164: 225–229, 1987
Del Principe D, Menichelli A, Mori PG, De Mattia D, Mancuso G, et al. Phase II trial of methylprednisolone pulse therapy in childhood chronic thrombocytopenia. Acta Haematologica (Basel) 77: 226–230, 1987
Derycke M, Dreyfus M, Ropert JC, Tchernia G. Intravenous immunoglobulin for neonatal isoimmune thrombocytopenia. Archives of Diseases in Childhood 60: 660–669, 1985
DiFino SM, Lachant NA, Kirshner JJ, Gottlieb AJ. Adult idiopathic thrombocytopenic purpura: clinical findings and response to therapy. American Journal of Medicine 69: 430–442, 1980
Dunn NL, Maurer HM. Prednisone treatment of acute idiopathic thrombocytopenic purpura of childhood. American Journal of Pediatric Hematology/Oncology 6: 159–164, 1984
Fehr J, Hofmann V, Kappeier U. Transient reversal of thrombocytopenia in idiopathic thrombocytopenic purpura by high-dose intravenous gammaglobulin. New England Journal of Medicine 306: 1254–1258, 1982
Fenaux P, Caulier MT, Hirschauer MC, Beuscart R, Goudemand J, et al. Reevaluation of the prognostic factors for splenectomy in chronic idiopathic thrombocytopenic purpura (ITP): a report on 181 cases. European Journal of Haematology 42: 259–264, 1989
Gernsheimer T, Stratton J, Ballem PJ, Slichter SJ. Mechanisms of response to treatment in autoimmune thrombocytopenic purpura. New England Journal of Medicine 320: 974–980, 1989
Gibson J, Richard KA, Bautovich G, May J, Kronenberg H. Management of splenectomy failures in chronic immune thrombocytopenic purpura: role of accessory splenectomy. Australian and New Zealand Journal of Medicine 16: 695–698, 1986
Guthrie Jr TH, Oral A. Immune thrombocytopenia purpura: a pilot study of Staphylococcal protein A immunomodulation in refractory patients. Seminars in Hematology 26 (Suppl. 1): 3–XX, 1989
Handin RI, Stossel TP. Effect of corticosteroid therapy on the phagocytosis of antibody-coated platelets by human leukocytes. Blood 51: 771–779, 1978
Hill W, Landgraf R. Successful treatment of amegakaryocytic thrombocytopenic purpura with cyclosporine. New England Journal of Medicine 312: 1060–1061, 1985
Hollenberg JP, Subak LL, Ferry Jr JJ, Bussel JB. Cost-effectiveness of splenectomy versus intravenous gamma globulin in treatment of chronic immune thrombocytopenic purpura in childhood. Journal of Pediatrics 112: 530–539, 1988
Hoyle C, Darbyshire P, Eden OB. Idiopathic thrombocytopenia in childhood: Edinburgh experience 1962–82. Scottish Medical Journal 31: 174–179, 1986
Ikkala E, Kivilaakso E, Kotilainen M, Hastbacka J. Treatment of idiopathic thrombocytopenic purpura in adults: long-term results in a series of 41 patients. Annals of Clinical Research 10: 83–86, 1978
Imbach P, Wagner HP, Berchtold W, Gaedicke G, Hirt A, et al. Intravenous immunoglobulin versus oral corticosteroids in acute immune thrombocytopenic purpura in childhood. Lancet 2: 464–468, 1985
Jacobs P, Wood L, Dent DM. Results of treatment in immune thrombocytopenia. Quarterly Journal of Medicine 58: 153–165, 1986
Jayabose S, Patel P, Inamdar S, Brilliant R, Mamtani R. Use of intravenous methylprednisolone in acute idiopathic thrombocytopenic purpura. American Journal of Pediatric Hematology/Oncology 9: 133–135, 1987
Jim RTS. Therapeutic use of colchicine in thrombocytopenia. Hawaii Medical Journal 45: 221–226, 1986
Joseph A, Evans DI. Immunosuppressive treatment of idiopathic thrombocytopenic purpura in children. Acta Paediatrica Scandinavica 71: 467–469, 1982
Kaplan C, Daffos F, Forestier F, Cox WL, Lyon-Caen D, et al. Management of alloimmune thrombocytopenia: antenatal diagnosis and in utero transfusion of maternal platelets. Blood 72: 340–343, 1988
Kelsey PR, Schofield KP, Geary CG. Refractory idiopathic thrombocytopenic purpura (ITP) treated with cyclosporine. Correspondence. British Journal of Haematology 60: 197–198, 1985
Kelton JG. Management of the pregnant patient with idiopathic thrombocytopenic purpura. Annals of Internal Medicine 99: 796–800, 1983
Kelton JG. The interaction of IgG with reticuloendothelial cells: biological and therapeutic implications. In Garratty G (Ed.) Current concepts in transfusion therapy, pp. 51–107, American Association of Blood Banks, Arlington, 1985
Kelton JG, Inwood MJ, Barr RM, Effer SB, Hunter D, et al. The prenatal prediction of thrombocytopenia in infants of mothers with clinically diagnosed immune thrombocytopenia. American Journal of Obstetrics and Gynecology 144: 449–454, 1982
Kelton JG, McDonald JW, Barr RM, Walker I, Nicholson W, et al. The reversible binding of vinblastine to platelets: implications for therapy. Blood 57: 431–438, 1981
Kim HC, Park CL, Cowan III JH, Fattori FD, August CS. Massive intravascular hemolysis associated with intravenous immunoglobulin in bone marrow transplant recipients. American Journal of Pediatric Hematology/Oncology 10: 69–74, 1988
Kim KS. Use of intravenous immunoglobulin in bacteria diseases. Annals of Internal Medicine 107: 369–371, 1987
Kitchen CS. Amelioration of endothelial abnormalities by prednisone in experimental thrombocytopenia in the rabbit. Journal of Clinical Investigation 60: 1129–1134, 1977
Kochupillai V, Nundy S, Sharma S. Idiopathic thrombocytopenic purpura in adults: response to corticosteroids and splenectomy. Journal of the Association of Physicians of India 34: 555–558, 1986
Königswieser H. Incidence of serious infections after splenectomy in children. Progress in Pediatric Surgery 18: 173–181, 1985
Krause JR. Chronic idiopathic thrombocytopenic purpura (ITP): development of acute non-lymphocytic leukemia subsequent to treatment with cyclophosphamide. Medical and Pediatric Oncology 10: 61–65, 1982
Lederman HM, Roifman CM, Lavi S, Gelfand EW. Corticosteroids for prevention of adverse reactions to intravenous immune serum globulin infusions in hypogammaglobulinemic patients. American Journal of Medicine 81: 443–446, 1986
Leroy J, Leclerc MH, Delahousse B, Guerois C, Foloppe P, et al. Treatment of heparin-associated thrombocytopenia and thrombosis with low molecular weight heparin (CY 216). Seminars in Thrombosis and Hemostasis 11: 326–329, 1985
Mazzucconi MG, Francesconi M, Fidani P, Di Nucci G, Gandolfo GM, et al. Treatment of idiopathic thrombocytopenic purpura (ITP): results of a multicentric protocol. Haematologica (Pavia) 70: 329–336, 1985
McMillan R, Longmire R, Yelenosky R. The effect of corticosteroids on human IgG synthesis. Journal of Immunology 116: 1592–1595, 1976
McMillan R, Longmire RL, Yelenosky R, Smith RS, Craddock CG, et al. Immunoglobulin synthesis in vitro by splenic tisue in idiopathic thrombocytopenic purpura. New England Journal of Medicine 286: 681–684, 1972
Mueller-Eckhardt C, Kiefel V. High-dose IgG for post-transfusion purpura-revisited. Blut 57: 163–167, 1988
Mueller-Eckhardt C, Salama A. Anti-Rh(D) immunoglobulin for immune thrombocytopenic purpura. Correspondence. Lancet 2: 50, 1986
Neppert J, Clemens M, Mueller-Eckhardt C. Immune phagocytosis inhibition by commercial immunoglobulins. Blut 52: 67–72, 1986
Nesher G, Dollberg L, Zimran A, Hershko C. Hepatosplenic peliosis after danazol and glucocorticoids for ITP. Correspondence. New England Journal of Medicine 312: 242, 1985
Oksenhendler E, Bierling P, Brossard Y, Schenmetzler C, Girard PM, et al. Anti-RH immunoglobulin therapy for human immunodeficiency virus-related immune thrombocytopenic purpura. Blood 71: 1499–1502, 1988
Oksenhendler E, Bierling P, Farcet JP, Rabian C, Seligmann M, et al. Response to therapy in 37 patients with HIV-related thrombocytopenic purpura. British Journal of Haematology 66: 491–495, 1987
O’Neal BJ, McDonald JC. The risk of sepsis in the asplenic adult. Annals of Surgery 194: 775–778, 1981
Pettersson T, Riska H, Nordström D, Honkanen E. Plasmapheresis for idiopathic thrombocytopenic purpura unresponsive to intravenous immunoglobulin. Correspondence. European Journal of Haematology 39: 92–93, 1987
Pizzuto J, Ambriz R. Therapeutic experience on 934 adults with idiopathic thrombocytopenic purpura: multicentric trial of the Cooperative Latin American Group on Hemostasis and Thrombosis. Blood 64: 1179–1183, 1984
Proctor SJ, Jackson G, Carey P, Stark A, Finney R, et al. Improvement of platelet counts in steroid-unresponsive idiopathic immune thrombocytopenic purpura after short-course therapy with recombinant α 2b interferon. Blood 74: 1894–1897, 1989
Quiquandon I, Fenaux P, Caulier MT, Pagniez D, Huart JJ, et al. Re-evaluation of the role of azathioprine in the treament of adult chronic idiopathic thrombocytopenic purpura: a report on 53 cases. British Journal of Haematology 74: 223–228, 1990
Rosse WF. Whatever happened to vinca-loaded platelets? New England Journal of Medicine 310: 1051–1502, 1984
Russo D, Gugliotta L, Mazzucconi MG, Isacchi G, Tonielli E, et al. Long-term results of splenectomy in adult chronic idiopathic thrombocytopenic purpura. Haematologica (Pavia) 72: 445–449, 1987
Salama A, Kiefel V, Amberg R, Mueller-Eckhardt C. Treatment of autoimmune thrombocytopenic purpura with Rhesus antibodies (anti-Rho (D)). Blut 49: 29–35, 1984
Salama A, Kiefel V, Mueller-Eckhardt C. Effect of IgG anti-Rho (D) in adult patients with chronic autoimmune thrombocytopenia. American Journal of Hematology 22: 241–250, 1986
Saleh M, Court W, Huster W, Shaw D, LoBuglio A. Effect of commercial immunoglobulin G preparation on human monocyte Fc-receptor dependent binding of antibody coated platelets. British Journal of Haematology 68: 47–51, 1988
Schreiber AD, Chien P, Tomaski A, Cines DB. Effect of danazol in immune thrombocytopenic purpura. New England Journal of Medicine 316: 503–508, 1987
Schroeder JO, Euler HH, Löffler H. Synchronization of plasma-pheresis and pulse cyclophosphamide in severe systemic lupus erythematosus. Annals of Internal Medicine 107: 344–346, 1987
Schwartz SI, Hoepp LM, Sachs S. Splenectomy for thrombocytopenia. Surgery 88: 497–506, 1980
Scott JR, Cruikshank DP, Kochenour NK, Pitkin RM, Warenski JC. Fetal platelet counts in the obstetric management of immunologic thrombocytopenic purpura. American Journal of Obstetrics and Gynecology 136: 495–499, 1980
Simon M, Jouet JP, Fenaux P, Pollet JP, Walter MP, et al. The treatment of adult idiopathic thrombocytopenic purpura: infusion of vinblastine in ITP. European Journal of Haematology 39: 193–196, 1987
Slichter SJ. Post-transfusion purpura: response to steroids and association with red blood cell and lymphocytotoxic antibodies. British Journal of Haematology 50: 599–605, 1982
Strother SV, Zuckerman KS, LoBuglio AF. Colchicine therapy for refractory idiopathic thrombocytopenic purpura. Archives of Internal Medicine 144: 2198–2200, 1984
Sultan Y, Kazatchkine MD, Maisonneuve P, Nydegger UE. Anti-idiotypic suppression of autoantibodies to factor VIII (anti-haemophilic factor) by high-dose intravenous gammaglobulin. Lancet 2: 765–768, 1984
Thomas DP. Reducing the risk of virus transmission of blood products. British Journal of Haematology 70: 393–395, 1988
Thompson RL, Moore RA, Hess CE, Wheby MS, Leavell BS, et al. Idiopathic thrombocytopenic purpura: long-term results of treatment and the prognostic significance of response to corticosteroids. Archives of Internal Medicine 130: 730–734, 1972
Van Hoff J, Ritchey AK. Pulse methylprednisolone therapy for acute childhood idiopathic thrombocytopenic purpura. Journal of Pediatrics 113: 563–566, 1988
Velu TJ, Debusscher L, Stryckmans PA. Ciclosporine for the treatment of refractory idiopathic thrombocytopenic purpura. Correspondence. European Journal of Haematology 38: 95, 1987
Verhoef GEG, Boonen S, Boogaerts MA. Ascorbate for the treatment of refractory idiopathic thrombocytopenia purpura. Correspondence. British Journal of Haematology 74: 234, 1990
Warkentin TE, Kelton JG. Heparin and platelets. Hematology-Oncology Clinics of North America 4: 243–264, 1990
Weinblatt ME, Kochen J, Ortega J. Danazol for children with immune thrombocytopenic purpura. American Journal of Diseases of Childhood 142: 1317–1319, 1988
Weinerman B, Maxwell I, Hryniuk W. Intermittent cyclophosphamide treatment of autoimmune thrombocytopenia. Canadian Medical Association Journal 111: 1100–1102, 1974
Wentz AC. Adverse effects of danazol in pregnancy. Annals of Internal Medicine 96: 672–673, 1982
Wood L, Jacobs P. Durable response to etoposide-loaded platelets in refractory immune thrombocytopenic purpura: a case report. American Journal of Hematology 27: 63–64, 1988
Woodruff RK, Grigg AP, Firkin FC, Smith IL. Fatal thrombotic events during treatment of autoimmune thrombocytopenia with intravenous immunoglobulin in elderly patients. Correspondence. Lancet 2: 217–218, 1986
Yoshida K, Wakui H, Mamiya S, Yamaguchi A, Miura AB. Bolus methylprednisolone therapy in adult idiopathic thrombocytopenic purpura. Japanese Journal of Medicine 26: 172–175, 1987
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Warkentin, T.E., Kelton, J.G. Current Concepts in the Treatment of Immune Thrombocytopenia. Drugs 40, 531–542 (1990). https://doi.org/10.2165/00003495-199040040-00004
Published:
Issue Date:
DOI: https://doi.org/10.2165/00003495-199040040-00004