Idiopathic Thrombocytopenic Purpura Treatment & Management
- Author: Michael A Silverman, MD, MD; Chief Editor: Gil Z Shlamovitz, MD, FACEP more...
Prehospital care focuses on the ABCs, which include providing oxygen, controlling severe hemorrhage, and initiating intravenous (IV) fluids to maintain hemodynamic stability. Airway control may be necessary for a large intracranial hemorrhage.
EMS providers should be aware of the potential for serious bleeding complications in patients with idiopathic thrombocytopenic purpura (ITP).
Emergency Department Care
Life-threatening bleeding requires conventional critical care interventions. In the patient with known ITP, high-dose parenteral glucocorticoids and IV immunoglobulin (IVIg), with or without platelet transfusions, are appropriate.
Platelet transfusion is indicated for controlling severe hemorrhage. Send a blood specimen to the lab for type and screen in case platelet transfusion is necessary. Platelet survival is increased if the platelets are transfused immediately after IVIg infusion. A consultation with a hematologist may be required to make a decision regarding the transfusion of platelets.
Guidelines for transfusion dosage are as follows:
6-8 U of platelet concentrate, or 1 U/10 kg
1 U of platelets to increase count of a 70-kg adult by 5-10,000/mm 3 and an 18-kg child by 20,000/mm 3
Splenectomy is reserved for patients in whom medical therapy fails. Emergent splenectomy is indicated in patients with life-threatening bleeding in whom medical therapy fails.
In patients without life-threatening complications, focus ED care on confirming the diagnosis, if possible, and initiating therapy as needed. Most patients with undiagnosed thrombocytopenia and purpura will need admission for further evaluation and treatment, since ITP is a diagnosis of exclusion.
Consult a hematologist for assistance in confirming the diagnosis or, in the patient with known ITP, arranging disposition and follow-up care, if appropriate.
Consult a neurosurgeon for intracranial hemorrhage. Consultation by other surgical specialists may be required for extensive hemorrhage at other sites.
Treatment in pediatric patients
American Society of Hematology (ASH) guidelines recommend that in children who have no bleeding, or only mild bleeding (ie, skin manifestations only, such as bruising and petechiae), regardless of the platelet count, management should be with observation alone. For pediatric patients who require treatment, recommended first-line agents are a single dose of intravenous immunoglobulin (IVIg; 0.8-1 g/kg) or a short course of corticosteroids; IVIg provides a more rapid increase in the platelet count. A single dose of anti-D immune globulin can be used in Rh-positive, nonsplenectomized children.
In children and adolescents who have significant ongoing bleeding despite first-line therapy, second-line treatments include rituximab or high-dose dexamethasone. Splenectomy is recommended for those who do not respond to, or cannot tolerate, other treatments and have significant or persistent bleeding, or need a better quality of life. However, ASH guidelines recommend waiting at least 12 months before performing splenectomy, in most cases.
Treatment in adults
A 2010 international consensus report advised that treatment for ITP is rarely indicated in adult patients with platelet counts above 50 × 109/L, in the absence of the following :
Bleeding due to platelet dysfunction or another hemostatic defect
Clearly identified comorbidities for bleeding
Mandated anticoagulation therapy
Profession or lifestyle that predisposes the patient to trauma
For adults with ITP, ASH guidelines recommend treating newly diagnosed patients whose platelet count is less than 30 × 109/L. Recommendations for first-line treatment include the following :
Longer courses of corticosteroids are preferred over shorter courses or treatment with IVIg (1 g/kg, one-time dose)
IVIg may be used with corticosteroids when a more rapid increase in platelet count is required
Either IVIg or anti-D (in appropriate patients) be used if corticosteroids are contraindicated
Recommended second-line treatments for cases that do not respond to corticosteroids, or recur afterward, are as follows:
Thrombopoietin receptor agonists for patients at risk of bleeding who relapse after splenectomy or who have a contraindication to splenectomy and have failed at least one other therapy
Rituximab may be considered for patients at risk of bleeding who have failed one line of therapy (eg, corticosteroids, IVIg, splenectomy)
Increasingly, clinicians are trying thrombopoietin receptor agonists before referring patients for splenectomy. According to published data, 25–30% of patients who are treated with thrombopoietin receptor agonists have a sustained response after stopping the drug. The mechanism for these sustained responses is not known. Spontaneous remission is unlikely, as the patients in reported studies had all received several prior treatments and had severe, prolonged thrombocytopenia.
Reese JA, Li X, Hauben M, Aster RH, Bougie DW, Curtis BR, et al. Identifying drugs that cause acute thrombocytopenia: an analysis using 3 distinct methods. Blood. 2010 Sep 23. 116(12):2127-33. [Medline]. [Full Text].
Warkentin TE, Anderson JA. DITP causation: 3 methods better than 1?. Blood. 2010 Sep 23. 116(12):2002-3. [Medline].
[Guideline] Neunert C, Lim W, Crowther M, Cohen A, Solberg L Jr, Crowther MA. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood. 2011 Apr 21. 117(16):4190-207. [Medline]. [Full Text].
Schultz CL, Mitra N, Schapira MM, Lambert MP. Influence of the American Society of Hematology guidelines on the management of newly diagnosed childhood immune thrombocytopenia. JAMA Pediatr. 2014 Oct. 168(10):e142214. [Medline].
Williams JA, Boxer LA. Combination therapy for refractory idiopathic thrombocytopenic purpura in adolescents. J Pediatr Hematol Oncol. 2003 Mar. 25(3):232-5. [Medline].
Pasa S, Altintas A, Cil T, Danis R, Ayyildiz O. The efficacy of rituximab in patients with splenectomized refractory chronic idiopathic thrombocythopenic purpura. J Thromb Thrombolysis. 2008 Mar 3. [Medline].
Khellaf M, Charles-Nelson A, Fain O, Terriou L, Viallard JF, Cheze S, et al. Safety and efficacy of rituximab in adult immune thrombocytopenia: results from a prospective registry including 248 patients. Blood. 2014 Nov 20. 124(22):3228-36. [Medline].
Rodeghiero F, Ruggeri M. Chronic immune thrombocytopenic purpura. New agents. Hamostaseologie. 2009 Jan. 29(1):76-9. [Medline].
Cersosimo RJ. Romiplostim in chronic immune thrombocytopenic purpura. Clin Ther. 2009 Sep. 31(9):1887-907. [Medline].
Kuter DJ, Bussel JB, Lyons RM, Pullarkat V, Gernsheimer TB, Senecal FM, et al. Efficacy of romiplostim in patients with chronic immune thrombocytopenic purpura: a double-blind randomised controlled trial. Lancet. 2008 Feb 2. 371(9610):395-403. [Medline].
Blanchette VS, Luke B, Andrew M, et al. A prospective, randomized trial of high-dose intravenous immune globulin G therapy, oral prednisone therapy, and no therapy in childhood acute immune thrombocytopenic purpura. J Pediatr. 1993 Dec. 123(6):989-95. [Medline].
Borst F, Keuning JJ, van Hulsteijn H, Sinnige H, Vreugdenhil G. High-dose dexamethasone as a first- and second-line treatment of idiopathic thrombocytopenic purpura in adults. Ann Hematol. 2004 Dec. 83(12):764-8. [Medline].
Cines DB, Blanchette VS. Immune thrombocytopenic purpura. N Engl J Med. 2002 Mar 28. 346(13):995-1008. [Medline].
El Alfy MS, Mokhtar GM, El-Laboudy MA, Khalifa AS. Randomized trial of anti-D immunoglobulin versus low-dose intravenous immunoglobulin in the treatment of childhood chronic idiopathic thrombocytopenic purpura. Acta Haematol. 2006. 115(1-2):46-52. [Medline].
Frederiksen H, Schmidt K. The incidence of idiopathic thrombocytopenic purpura in adults increases with age. Blood. 1999 Aug 1. 94(3):909-13. [Medline].
George JN, el-Harake MA, Raskob GE. Chronic idiopathic thrombocytopenic purpura. N Engl J Med. 1994 Nov 3. 331(18):1207-11. [Medline].
Heegaard ED, Rosthoj S, Petersen BL, et al. Role of parvovirus B19 infection in childhood idiopathic thrombocytopenic purpura. Acta Paediatr. 1999 Jun. 88(6):614-7. [Medline].
Longhurst HJ, O'Grady C, Evans G, et al. Anti-D immunoglobulin treatment for thrombocytopenia associated with primary antibody deficiency. J Clin Pathol. 2002 Jan. 55(1):64-6. [Medline].
Maloisel F, Andres E, Zimmer J. Danazol therapy in patients with chronic idiopathic thrombocytopenic purpura: long-term results. Am J Med. 2004 May 1. 116(9):590-4. [Medline].
McMillan R, Durette C. Long-term outcomes in adults with chronic ITP after splenectomy failure. Blood. 2004 Aug 15. 104(4):956-60. [Medline].
Newman GC, Novoa MV, Fodero EM. A dose of 75 microg/kg/d of i.v. anti-D increases the platelet count more rapidly and for a longer period of time than 50 microg/kg/d in adults with immune thrombocytopenic purpura. Br J Haematol. 2001 Mar. 112(4):1076-8. [Medline].
Ojima H, Kato T, Araki K. Factors predicting long-term responses to splenectomy in patients with idiopathic thrombocytopenic purpura. World J Surg. 2006 Apr. 30(4):553-9. [Medline].
Rodeghiero F. First-line therapies for immune thrombocytopenic purpura: re-evaluating the need to treat. Eur J Haematol Suppl. 2008 Feb. 19-26. [Medline].
Sandler SG. Intravenous Rh immune globulin for treating immune thrombocytopenic purpura. Curr Opin Hematol. 2001 Nov. 8(6):417-20. [Medline].
Sukenik-Halevy R, Ellis MH, Fejgin MD. Management of immune thrombocytopenic purpura in pregnancy. Obstet Gynecol Surv. 2008 Mar. 63(3):182-8. [Medline].
Thude H, Gruhn B, Werner U. Treatment of a patient with chronic immune thrombocytopenic purpura with rituximab and monitoring by flow cytometric analysis. Acta Haematol. 2004. 111(4):221-4. [Medline].
Vranou M, Platokouki H, Pergantou H, Aronis S. Recurrent idiopathic thrombocytopenic purpura in childhood. Pediatr Blood Cancer. 2008 Apr 17. [Medline].
Watts RG. Idiopathic thrombocytopenic purpura: a 10-year natural history study at the childrens hospital of alabama. Clin Pediatr (Phila). 2004 Oct. 43(8):691-702. [Medline].
Zeller B, Helgestad J, Hellebostad M. Immune thrombocytopenic purpura in childhood in Norway: a prospective, population-based registration. Pediatr Hematol Oncol. 2000 Oct-Nov. 17(7):551-8. [Medline].
Provan D, Newland AC. Current Management of Primary Immune Thrombocytopenia. Adv Ther. 2015 Oct. 32 (10):875-87. [Medline]. [Full Text].
Provan D, Stasi R, Newland AC, Blanchette VS, Bolton-Maggs P, Bussel JB, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010 Jan 14. 115 (2):168-86. [Medline]. [Full Text].