Although the pathophysiology of ITP is unclear, several mechanisms have been proposed such as autoantibodies and cytotoxic T cells that target platelets and/or megakaryocytes.1 Immune thrombocytopenia affects people of both sexes and all ages, with a peak in childhood and a second and third peak in young adults and the elderly.3 In the United States, the incidence of ITP is approximately 2 to 5 per 100,000 patients per year.2
Platelets are critical to the maintenance of vascular integrity; thus, patients with ITP are at increased risk for bruising and spontaneous bleeding events.1 Patients with a platelet count of <30×109/L are at increased risk for severe bleeding (eg, intracranial hemorrhage, mucocutaneous bleeding, lower gastrointestinal bleeding, other internal bleeding, and menorrhagia).4 Patients with a platelet count of <10×109/L are at risk for life-threatening bleeding.
Deaths associated with ITP are rare and associated most frequently with spontaneous or trauma-induced intracranial hemorrhage.4 Intracranial hemorrhage reportedly occurs in 1.4% of adults and 0.4% of children with ITP, most of whom have chronic ITP.5 If severe bleeding occurs, rapid intervention with platelet transfusions in conjunction with continuous infusion of intravenous immunoglobulin G (IgG) therapy or emergent splenectomy may be lifesaving.6
The signs and symptoms of ITP are highly variable, and one-quarter of patients are asymptomatic and diagnosed through routine blood tests.4 Symptoms primarily are related to bleeding in the skin or mucous membranes presenting as petechiae or purpura.6 Patients with platelet counts of ≥30×109/L usually do not require any treatment and can be managed with observation (Table 3).2 If adult patients require intervention, American Society of Hematology (ASH) guidelines recommend treatment with corticosteroids, which prevent the destruction of platelets by macrophages within the spleen.2 The ASH guidelines also include treatment recommendations for children.2
The prognosis of ITP is variable and appears to depend on age, with 1-year spontaneous remission rates in pediatric patients ranging from 74% in children younger than 1 year to 62% in patients aged 10 to 20 years.2 Spontaneous remission rates in adults range from 20% to 45% at 6 months.2
The patient in this case study was referred to the emergency department in response to her critical low platelet count. There the patient received a blood transfusion and high-dose steroids and was admitted to the hospital. The platelet count responded to therapy and began to rise. She was discharged with a platelet count of 38×109/L and diagnosed with ITP. She was sent home on oral steroids and advised to follow up with hematology. At her follow-up appointment with the hematologist, repeat laboratory studies revealed a decline in the platelet count to 3×109/L. This resulted in a second admission to the hospital. She received IgG therapy and, subsequently, the platelet count rose to 93×109/L. She received a total of 2 IgG treatments with good response, and there were no future treatments planned at that time. She moved back home, and no further updates were made.
Elizabeth Houser, CRNP, is a nurse practitioner at Indiana University of Pennsylvania, in Indiana, PA. She holds an MS in Nursing from Carlow University. Houser has been practicing in college health for 8 years and has an additional 5 years of emergency nursing experience.
1. Arnold DM. Bleeding complications in immune thrombocytopenia. Hematology. 2015:237-242. doi: 10.1182/asheducation-2015.1.237
2. Neunert C, Terrell DR, Arnold DM, et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv. 2019;3(23):3829-3866. doi: 10.1182/bloodadvances.2019000966
3. European Group for Blood and Marrow Transplantation. Immune thrombocytopenia: a practical guide for nurses and other allied healthcare professionals. J Thromb Haemost. 2015;13(3):457-64. doi: 10.1111/jth.12813
4. Neunert C, Noroozi N, Norman G, et al. Severe bleeding events in adults and children with primary immune thrombocytopenia: a systematic review. J Thromb Haemost. 2015;13(3):457-464. doi: 10.1111/jth.12813
5. Lambert MP, Gernsheimer TB. Clinical updates in adult immune thrombocytopenia. Blood. 2017;129(21):2829-2835. doi:10.1182/blood-2017-03-754119
6. Neunert C, Lim W, Crowther M, et al. The American Society of Hematology 2011 evidence-based practice guidelines for thrombocytopenia. Blood. 2011;117 (16): 4190-4207. doi:10.1182/blood-2010-08-302984