Idiopathic Thrombocytopenic Purpura Clinical Presentation
- Author: Michael A Silverman, MD, MD; Chief Editor: Gil Z Shlamovitz, MD, FACEP more...
Focus on the symptoms of bleeding (eg, type, severity, duration) and on symptoms that may exclude other causes of thrombocytopenia. Elicit risk factors for HIV and systemic symptoms linked to other illnesses or to medications (eg, heparin, alcohol, quinidine/quinine, sulfonamides) that may cause thrombocytopenia.
Medications can be a common etiology for inducing thrombocytopenia, and patients should have their medications carefully reviewed. One study used three distinct methods to document drugs that may be associated with drug-induced immune thrombocytopenia (DITP).[1, 2] Approximately 1500 drugs are associated with thrombocytopenia, but, using this analysis, 24 drugs had evidence of causing thrombocytopenia by all three methods.
Address risk factors for increased bleeding, such as GI disease, CNS disease, urologic disease, or active lifestyle, as these may determine the aggressiveness of management.
Common signs, symptoms, and precipitating factors include the following:
Abrupt onset (childhood ITP)
Gradual onset (adult ITP)
Recent live virus immunization (childhood ITP)
Recent viral illness (childhood ITP)
Limited data are available on the recurrent form of the disease. One study showed a 6% prevalence of recurrent ITP with most patients (69%) having only one recurrence. Though one third of patients had their recurrent episode within 3 months of their initial one, the remainder of patients had at least a 3-month interval between episodes.
Evaluate the type and the severity of bleeding and try to exclude other causes of bleeding. Seek evidence of liver disease, thrombosis, autoimmune diseases (eg, nephritis, cutaneous vasculitis, arthritis), and infection, particularly HIV.
Common physical findings include the following:
Nonpalpable petechiae, which mostly occur in dependent regions
Hemorrhagic bullae on mucous membranes
Signs of GI bleeding
Evidence of intracranial hemorrhage, with possible neurologic symptoms
Nonpalpable spleen: The prevalence of palpable spleen in patients with ITP is approximately the same as that in the non-ITP population (ie, 3% in adults, 12% in children).
Spontaneous bleeding when platelet count is less than 20,000/mm 3.
See the list below:
Immunoglobulin G (IgG) autoantibodies on the platelet surface
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].