الفهرس 
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Abstract
Childhood immune thrombocytopenia (ITP, previously known as idiopathic thrombocytopenic purpura) generally presents with the sudden appearance of a petechial rash, bruising, and/or bleeding in an otherwise healthy child.
• In about 44 percent of children with ITP, there is a history of a prior infection. Otherwise, the history does not reveal any significant findings.
• The examination of the child with suspected ITP generally reveals signs of cutaneous bleeding (petechiae, purpura, and ecchymoses). About 70 percent of patients also have mucosal bleeding (involving the nasal passages, oral cavity, and genitourinary and gastrointestinal tracts). Patients with platelet counts below 10,000/micro L are more likely to have mucosal bleeding.
• Serious hemorrhage requiring blood transfusions occurs in about 2 percent of children with ITP. Intracranial hemorrhage is the most severe consequence of ITP and is rare, with a reported incidence of 0.1 to 0.8 percent( Neunert et al. , 2015 ).
• About 96 percent of affected children have the newly-diagnosed form of ITP, defined as a complete remission (platelet count >150,000/micro L) within three months of presentation. Children with chronic ITP (thrombocytopenia lasting more than 12 months after presentation) are more likely at presentation to have an insidious onset of symptoms, be older, and not have a history of a prior infection compared to those with acute ITP.
• The diagnosis of ITP is based upon the following :
Isolated thrombocytopenia (platelet count <100,000/micro L), with otherwise normal blood counts and peripheral blood smear.
No clinically apparent associated conditions that may cause thrombocytopenia. In particular, a disorder other than ITP is suggested if there is enlargement of lymph nodes, liver, or spleen, systemic symptoms (eg, fever, anorexia, bone or joint pain, or weight loss) or a prior history of atypical bleeding or significant disease.
The initial laboratory evaluation includes a complete blood count (CBC) and peripheral blood smear, with reticulocyte count, direct antiglobulin test (DAT), and immunoglobulin levels (table 1). Any findings that are inconsistent with ITP (eg, abnormal white blood cell count or blood smear) should lead to evaluation for another cause of thrombocytopenia.
A bone marrow examination is not required in children with a typical presentation and laboratory findings of ITP. Indications for bone marrow aspiration and biopsy include atypical clinical or laboratory features at presentation, new findings that emerge during follow-up that are not consistent with ITP, or failure to respond to treatment if treatment is given.
For patients with laboratory abnormalities other than thrombocytopenia or with clinical features that are not typical of ITP, other disorders that can cause thrombocytopenia should be excluded. These include malignancy, infection, autoimmune disorders, drug-induced thrombocytopenia, and bone marrow failure . The steps in the further evaluation depend on the reason for concern.
Depending on individual patient characteristics, appropriate initial management of newly diagnosed ITP may be either watchful waiting or pharmacologic intervention. Management is not standardized, and treatment decisions incorporate multiple considerations including the patient’s presenting characteristics and the patient and family’s values and preferences, as well as provider comfort and experience.
The main options for initial pharmacologic intervention include glucocorticoids, intravenous immune globulin (IVIG), or intravenous anti-D immune globulin (anti-D, also known as anti-Rho immune globulin). These agents will raise the platelet count more quickly than no therapy. The decision among these options should be individualized and depends on urgency (IVIG and anti-D achieve a more rapid rise in platelets than glucocorticoids), cost, and need for monitoring for adverse effects .
For children with ITP with severe or life-threatening bleeding (eg, severe or prolonged epistaxis, gastrointestinal bleeding, or intracranial hemorrhage [ICH]) we recommend both platelet transfusions and pharmacologic therapy . For these patients, we suggest combination pharmacotherapy, usually consisting of high-dose glucocorticoids and IVIG, with or without anti-D.
We also suggest pharmacologic intervention for children with ITP who have one or more factors that increase the likelihood of bleeding risk . Important risk factors that prompt pharmacotherapy include :
Severe thrombocytopenia (platelets <10,000/micro L) with signs of substantial cutaneous bleeding (extensive bruising, bleeding, or petechiae)
Moderate thrombocytopenia (platelets <20,000/micro L) with mucosal bleeding
Past or anticipated factors that increase bleeding risk (such as recent head trauma) For such patients, either IVIG, anti-D, or a short course of glucocorticoids are appropriate first-line treatment options. However, treatment response is not fully predictable, and careful monitoring is required.
For children with no bleeding or mild bleeding (defined as skin manifestations only, such as bruising and petechiae) and without the risk factors listed above, we suggest no pharmacologic intervention (”watchful waiting”) . Other factors that may influence decisions about pharmacotherapy include the patient’s acceptance of activity restriction, quality of life, and assurance of close follow-up.
Whether or not pharmacologic therapy is used, medications with either antiplatelet or anticoagulant activity should be avoided until the thrombocytopenia has resolved. In addition, patients with moderate or severe thrombocytopenia should restrict activities with a risk for trauma, including contact sports. The nature and duration of the activity restriction should be individualized and made in collaboration with the patient and family, and may inform decisions about pharmacotherapy.
The great majority of children with ITP are managed in the ambulatory setting, including those who receive pharmacologic intervention. However, some patients may be hospitalized for one to three days for treatment and/or monitoring. In the ambulatory setting, platelet counts are initially monitored once weekly. When recovery of platelet counts is detected, the interval between measuring platelet counts may be lengthened, but monitoring should continue until the platelet count has returned to normal (>150,000/micro L) and is stable without treatment.