Presentation, Diagnosis, and Nursing Management

TON - September 2012 VOL 5, NO 8 — October 12, 2012

Idiopathic thrombocytopenic purpura (ITP), now more commonly referred to as immune thrombocytopenia, is defined as a hemorrhagic disorder in which there is a pronounced reduction in circulating blood platelets due to the presence in blood plasma of a substance that agglutinates platelets.1 Despite this definition, the risk of bleeding in patients with ITP seems dis proportionately low, compared with the circulating platelet counts.

ITP Presentation and Diagnosis
Patients may be diagnosed incidentally with a low platelet count (<100,000), but they may be asymptomatic. Others may suffer catastrophic bleeding prior to seeking medical attention and may be hospitalized, with significant morbidity and mortality. The challenge for clinicians is to identify the patients who are at the highest risk of bleeding, which increases morbidity and mortality. There are no good epidemiological studies that de fine the incidence or prevalence of ITP. Separate studies have estimated the incidence of secondary ITP, such as with MMR (measles, mumps, rubella) vaccination; a review of 11 studies estimated the incidence of ITP at 2.6 cases per 100,000 from the vaccine.2 A Mary land study estimated the prevalence of secondary ITP in that state at 9.5 per 100,000 persons, based on claims submitted for privately insured patients.3

Signs and symptoms of bleeding secondary to thrombocytopenia include petechial rash (fine red pinpoint rash over the lower extremities or at pressure points such as the waistband, sock line, etc) due to bleeding from the small capillaries; purpura, bleeding under the skin or in the mucous membranes, typically larger than petechiae; increased bruising or ecchymosis, especially in an area that does not suffer trauma, such as the inner aspect of the arms or thighs or on the torso; epistaxis (nosebleed) that does not stop easily or is recurrent outside of the patient’s normal experience; and gingival bleeding along the gums with or without trauma from brushing or flossing. Pertinent neg atives to note during a review of systems and physical examination include no hepatosplenomegaly; no lymph adenopathy; and no reports of weight loss, bone pain, or night sweats. Primary ITP occurs in isolation. Ob jective and subjective findings of a more involved process would require further diagnostic workup for secondary ITP, including a bone marrow aspirate and biopsy. This procedure is no longer recommended for diagnosis of primary ITP in children or adults younger than 60 years.4

Secondary ITP, thrombocytopenia that occurs due to another autoimmune process, can occur in children and adults. In children, viral infections or vaccinations (MMR) may be implicated in the development of ITP. Drug therapy or infection (HIV, hepatitis C, Helicobacter pylori) can precipitate ITP in adults. Studies have shown that the eradication of H pylori can improve the platelet count in children with ITP.5 Secondary ITP may be due to increased platelet destruction by circulating antibodies, sequestration in the spleen, or other processes. Treatment of thrombocytopenia will be dependent on not only the signs and symptoms associated with it but also the underlying problem, such as viral infection.

The clinician assessing a patient with thrombocytopenia will evaluate the complete blood count with differential and platelets. The white blood cell count should be normal within the expected range of white blood cells. Red blood cell morphology should be normal; anemia is expected if bleeding has occurred but not in the absence of bleeding. Red blood cell indexes (MCV, MCHC, RDW, MCH) should be normal. Thrombocytopenia should be an isolated finding on the complete blood count. The peripheral smear should be reviewed for clumping of platelets, which may cause the automated counter to report a lower number of circulating platelets. Platelets will be decreased in number (<100,000, often <20,000), with normal to large-sized platelets.

Childhood ITP typically occurs in children 2 to 10 years old. Spontaneous remission occurs in 85% of the patients, usually within 6 weeks but may take up to 12 months.6 Depending on the signs and symptoms of thrombocytopenia, careful monitoring without intervention is reasonable.

The prognosis for adolescents and adults with ITP differs from that for children, as spontaneous remission is much less likely, occurring in 22% to 30% of patients older than 10 years. This population is more likely to require long-term management.6

Nursing Management of the Patient With ITP Although thrombocytopenia is associated with increased bleeding, it is not uncommon for patients with ITP to have no bleeding despite platelet counts <20,000. Frequently, patients with ITP are followed by hematology specialists and cared for by oncology nurses. It is important to recognize that platelet transfusion remains the primary method for managing active, life-threatening bleeding. However, transfusing platelets without evidence of bleeding is not encouraged. Patients with ITP should be assessed for bleeding at each clinic visit. Proper assessment of the patient includes a thorough history and physical.7 Historical reports of bleeding episodes can help guide future preventive measures and avoid significant morbidity. A patient with a history of peptic ulcer disease should take preventive medications to prevent recurrence of this disease in the presence of thrombocytopenia. Patient education and management should in clude avoiding over-the-counter medications that could interfere with hemostasis. As pirin alternative medications for the management of pain may need to be prescribed if the patient typically uses nonsteroidal anti-inflammatory medications. Women who are experiencing heavy or prolonged menstrual cycles may require hormonal therapy to suppress menses or to induce amenorrhea.8

Patient understanding of the treatment regimen, including dosing and timing of medications, is essential to successful management. Providing verbal and written instructions regarding medications and regimens decreases confusion and improves patient adherence to the overall treatment plan. Some frontline therapies, such as steroids, can increase the risk of bleeding due to changes in the mucosal lining of the stomach and duodenum.9 It is essential that patients be given the information and tools to ameliorate their risk of bleeding while undergoing treatment. Nurses spend the most time with patients overall and are in the best position to reinforce the treatment plan to ensure the best outcome. Patients are required to master complex drug therapy and treatment schedules; thus, one of the best tools to give them at their first visit is access to care. Providing a telephone number where a healthcare professional can be reached for questions, concerns, or emergencies is essential to prevent significant morbidity and mortality.

 

A 3-year-old ITP patient with platelet count of 3 K/μL.    Image courtesy of John Lazarchick, MD.
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References

  1. Taber CW, Thomas CL. Taber’s Cyclopedic Medical Dictionary. 18th ed. Philadelphia, PA: F.A. Davis; 1997.
  2. Neunert C, Lim W, Crowther M, et al. The American Society of Hematology 2011 evidence based practice guideline for immune thrombocytopenia. Blood. 2011;117(16):4190-4207.
  3. Segal JB, Powe NR. Prevalence of immune thrombocytopenia: analyses of administrative data. J Thromb Haemost. 2006;4(11):2377-2383.
  4. Jubelirer SJ, Harpold R. The role of the bone marrow examination in the diagnosis of immune thrombocytopenic purpura: case series and literature review. Clin Appl Thromb Hemost. 2002;8(1):73-76.
  5. Neefjes VM, Heijboer H, Tamminga RY. H. pylori infection in childhood chronic immune thrombocytopenic purpura. Haematologica. 2007;92(4):576.
  6. Kühne T, Buchanan GR, Zimmerman S, et al. A prospective comparative study of 2540 infants and children with newly diagnosed idiopathic thrombocytopenic purpura (ITP) from the Intercontinental Childhood ITP Study Group. J Pediatr. 2003;143(5):605-608.
  7. Friend PH, Pruett J. Bleeding and thrombotic complications. In: Yarbro CH, Frogge MH, Goodman M, eds. Cancer Symptom Management. 3rd ed. Sudbury, MA: Jones & Bartlett; 2004:233-251.
  8. Damron BI, Samsonow SM. Prevention of bleeding. In: Eaton LH, Tipton JM, eds. Putting Evidence Into Practice: Improving Oncology Patient Outcomes. Pittsburgh, PA: Oncology Nursing Society; 2009:253-265.
  9. Hernández-Diaz S, Rodríguez LA. Steroids and risk of upper gastrointestinal complications. Am J Epidemiol. 2001;153(11):1089-1093.

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