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I.

INTRODUCTION

Idiopathic thrombocytopenic purpura (ITP), also known as primary immune thrombocytopenic purpura is hemorrhagic disorder of the blood and an acquired disease of children and adults, defined as isolated thrombocytopenia with no clinically apparent associated conditions or other causes of thrombocytopenia. ITP is a disorder in which antibodies are generated against platelet antigens, resulting in accelerated platelet destruction. It is characterized by severe acquired thrombocytopenia and mucocutaneous bleeding due to opsonization and destruction of platelets by circulating antibody. ITP may occur in isolation (primary) or in association with other disorders (secondary).

In 1735, Paul Gottlieb, a German physician, first described ITP and was therefore known as Werlhofs disease (Nakhoul, Kozuc, Varma, 2006). In 1916, Paul Kaznelson reported the first successful treatment for ITP after a patient showed a response to splenectomy (Kaznelson, 1916). Splenectomy was then used as the first-line therapy for ITP until 1950. In 1951, William J Harrington and James W Hollingsworth established that ITP was an autoimmune condition (Harrington, Minnich, Hollingsworth, et al. 1951). They postulated that the destruction of platelets in ITP was caused by a factor circulating in blood. Their experiment included Harrington receiving blood from an ITP patient which within 3 hours resulted in his platelet count dropping to a seriously low level, causing a seizure. It took 5 days for his platelet count to return to normal levels. Antibodies, usually immunoglobulin G, specific to platelet membrane glycoproteins (GPIIb-IIIa complex is the most common) have since been identified as the circulating blood factor involved in the destruction of platelets in ITP (Tomer, Koziol, McMillan, 2005; Li, Yang, Xia, et al. 2001; Fujisawa, Tani, OToole, et al. 1992; Shulman, Marder, Weinrach, 1965; Harrington, Sprague, Minnich, et al. 1953).

The American Society of Hematology defined ITP as isolated thrombocytopenia with no clinically apparent associated conditions or other causes of thrombocytopenia

and noted that no specific criteria establish the diagnosis of ITP. A later guideline from the British Society of Hematology was more explicit in defining the syndrome, defining it as an autoimmune disorder characterized by persistent thrombocytopenia (peripheral blood platelet count (less than 150 x 109/L) due to autoantibody binding to platelet antigens causing their premature destruction by the reticuloendothelial system, and in particular the spleen.

As reflected by major society practice guidelines, the diagnosis of ITP essentially rests on the finding of thrombocytopenia that is presumed to be immune-mediated after other causes (such as hypoproduction or mechanical destruction) have been excluded. However, the definition of thrombocytopenia has also varied, with past definitions for platelet counts among studies ranging from 50,000 to 150,000, complicating the interpretation of these reports. Patients with platelet counts between 100 and 150 who otherwise met the diagnosis criteria for ITP did well, with only 7% developing more severe disease, and the remainder either improving or remaining stable. This has led some authors to state that it may be inappropriate to apply the diagnosis to patients in this group. Furthermore, the commonly used reference ranges observed in populations of European descent have been found to be higher than those in several other ethnic groups, suggesting that platelet levels below 150,000 cell/L may be normal in these populations. These issues are addressed in part by the recommendations from the international working group report, in that while recognizing that variability in the normal platelet count has been demonstrated among different populations, the recently proposed international consensus statement recommends defining thrombocytopenia as <100,000 platelets per microliter.

Given the evolving definition of ITP, prior estimates of the incidence and prevalence of the disorder have varied. Nonetheless, the existing data for primary ITP can provide some insight into the frequency of the disorder. In the U.S., the estimated annual incidence of ITP is approximately 1 per 10,000 to 1 per 1,000 persons. There are about 100 new cases per million each year, with about half of those cases developing in children.

Women develop ITP more commonly than men, with a female to male ratio of 2-3:1. In children, the ratio is about even. It affects all ages and ethnic groups. Currently, there are around 200,000 individuals with ITP in the U.S. Meanwhile, in the Northern European populations, estimates of the overall incidence of ITP in adults range from 1.6 to 3.9 patients per 100,000 person-years, whereas estimates of prevalence range widely from 9.5 to as high as 189.3 (Landgren et. al) per 100,000 population. In a recent study employing the UK-based General Practice Research Database, adult ITP was estimated to have an incidence of 4.4 per 100,000 person-years in women and 3.4 per 100,000 person-years in men, with gender specific differences in incidence rates being most prominent in persons under 65 years of age. Clearly, differences among data sources and definitions among studies may be playing a role in the observed variability among reports, highlighting the need for further studies using more stringently defined disease criteria.

Differences in both host factors and extrinsic risk factors for ITP exist in children relative to those in adults. Notably, the female preponderance in ITP cases that is seen in adults is not seen in pediatric populations. In children, a majority of ITP cases have been associated with viral infections, with demonstrable seasonal variations in incidence. Additionally, the natural history of the disease differs between adult and childhood ITP in that childhood ITP progresses to chronic disease much more rarely. ITP in young children may take an acute, self-limited course, whereas chronic forms of ITP may be more common in children older than age 10.

By choosing ITP as a case study, the nurse researcher expects to broaden his knowledge, understanding, and management of this blood disorder. It is imperative for todays nurses to be adequately informed regarding the knowledge and skill in the management of this condition since almost 60 years after its recognition, ITP remains a poorly studied and under-recognized disease. It would, unquestionably help the nurse for when in the actual setting; such disorders would require a huge grasp in order to provide

optimal health care. Equipped with the true knowledge of this condition, a high quality of care will be provided to those people suffering from it.

OBJECTIVES Nurse Centered: General: After establishing a nurse-patient interaction and careful study of the patients condition, the nurse researcher will be able to gain knowledge, develop skills and enhance attitude to determine appropriate care and management of patients with Idiopathic Thrombocytopenic Purpura.

Specific: Generate a comprehensive presentation of the disease condition by means of correct presentation of the data gathered through the use of nursing process; Present the current trends about the disease condition, reason for choosing such case for presentation, and the importance of the case study; Formulate a comprehensive nursing care plan to meet the patients needs to promote and facilitate patients recovery; Provide health teachings to the patient and his family in relation to the patients needs in response to the disease process and therapy; and Evaluate the result of the patients response to medical and nursing management.

Patient Centered: General: After establishing a nurse-patient interaction and careful study of the patients condition, the patient and his significant others shall have an increased awareness about the disease and will establish self-reliance with regards to the measurements needed and proper intervention for his condition.

Specific: Build a trusting relationship with the nurse as well as the other members of the health care team;

Gain knowledge on the definition of ITP, its causes, risk factors, possible complications and prevention/supportive management; and

Render proper nursing management and medical regimen needed by the patient.