idiopathic thrombocytopenic purpura
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Idiopathic Thrombocytopenic Purpura
Causes and symptoms
- female sex
- age over 10 years at onset of symptoms
- slow onset of bruising
- presence of other autoantibodies in the blood
General care and monitoring
Medications and transfusions
There are two general types of thrombocytopenic purpura: primary or idiopathic, in which the cause is unknown, and secondary or symptomatic, which may be associated with exposure to drugs or other chemical agents, systemic diseases such as multiple myeloma and leukemia, diseases affecting the bone marrow or spleen, and infectious diseases such as rubella (German measles).
id·i·o·path·ic throm·bo·cy·to·pe·nic pur·pu·ra (ITP),
idiopathic thrombocytopenic purpuraWerlhof's disease Hematology A diagnosis of exclusion characterized by thrombocytopenia without known exogenous etiologic factors, or conditions known to be associated with 'secondary' thrombocytopenia; in most Pts, ITP is attributed to immune-mediated accelerated platelet destruction; acute ITP is more common in children, causing a self-limited wave of ecchymotic hemorrhage 2º to viral infection or vaccination; chronic ITP is more common in adults and often is autoimmune with mucocutaneous, CNS, cardiac and renal hemorrhage–and potentially, infarction, bruisability, transient thrombocytopenia–with normal or ↑ megakaryocytes in BM;2⁄3 of cases have IgG antiplatelet antibodies and hemolysis in splenic sinusoids Clinical ♀:♂ ratio 3:1, microangiopathic hemolytic anemia, fever, transient neurologic defects, renal failure, microthrombolic 'showers' to brain, heart, lungs, kidneys, adrenal glands, spleen and liver DiffDx Microthrombi of TTP of young ♀, microangiopathic hemolytic anemia, neurologic defects, renal failure Treatment Efficacy of most therapies used in ITP is uncertain, especially given that spontaneous remission occurs, the small populations studied, and the wide difference in Pt populations; modalities include splenectomy–up to 75% markedly improve with splenectomy, prednisone, IV gamma globulin, immunoadsorption apheresis on staphylococcal protein A columns, plasmapheresis; low-dose corticosteroids or to pulsed high-dose dexamethasone, combination chemotherapy–eg, azathioprine, cochicine, cyclosporine, danazol, vincristine
id·i·o·path·ic throm·bo·cy·to·pe·nic pur·pu·ra(ITP) (id'ē-ō-path'ik throm'bō-sī-tō-pē'nik pŭr'pyŭr-ă)
Synonym(s): immune thrombocytopenic purpura, purpura hemorrhagica, thrombopenic purpura.
idiopathic thrombocytopenic purpuraAbbreviation: ITP
CAUTION!People with ITP should take special precautions to avoid injuries in contact sports. Aspirin and other drugs that may cause bleeding should only be taken by people with ITP under direction of an experienced physician.
Symptoms may include bleeding from the nose, the gums, or the gastrointestinal tract. Physical findings include petechiae, esp. on the lower extremities, and ecchymoses. Laboratory findings: The platelet count is usually less than 20,000/mm3, bleeding time is prolonged, and may be associated with mild anemia as a result of bleeding.
If patients are asymptomatic (i.e., have no active bleeding) and have platelet counts of about 50,000/mm3, treatment is not needed (4 out of 5 patients recover without treatment). Treatment for symptomatic patients, or patients with very low platelet counts, usually is with glucocorticoids or immune globulin for acute cases and corticosteroids for chronic cases. For those who do not respond within 1 to 4 months, treatment may include high-dose corticosteroids, intravenous immune globulin (IVIG), immunosupression, immunoabsorption apheresis using staphylococcal protein-A columns to filter antibodies out of the bloodstream, AntiRhD therapy for those with specific blood types, splenectomy, or chemotherapeutic drugs such as vincristine or cyclophosphamide.
Platelet count is monitored closely. The patient is observed for bleeding (petechiae, ecchymoses, epistaxis, oral mucous membrane or GI bleeding, hematuria, menorrhagia) and stools, urine, and vomitus are tested for occult blood. The amount of bleeding or size of ecchymoses is measured at least every 24 hr. Any complications of ITP are monitored. The patient is educated about the disorder, prescribed treatments, and importance of reporting bleeding (such as epistaxis, gingival, urinary tract, or uterine or rectal bleeding) and signs of internal bleeding (such as tarry stools or coffee-ground vomitus). The patient should avoid straining during defecation or coughing because both can lead to increased intracranial pressure, possibly causing cerebral hemorrhage. Stool softeners are provided as necessary to prevent tearing of the rectal mucosa and bleeding due to passage of constipated or hard stools. The purpose, procedure, and expected sensations of each diagnostic test are explained. The role of platelets and the way in which the results of platelet counts can help to identify symptoms of abnormal bleeding are also explained. The lower the platelet count falls, the more precautions the patient will need to take; in severe thrombocytopenia, even minor bumps or scrapes can result in bleeding. The nurse guards against bleeding by taking the following precautions to protect the patient from trauma: keeping the side rails of the bed raised and padded, promoting use of a soft toothbrush or sponge-stick (toothette) and an electric razor, and avoiding invasive procedures if possible. When venipuncture is unavoidable, pressure is exerted on the puncture site for at least 20 min or until the bleeding stops. During active bleeding, the patient maintains strict bedrest, with the head of the bed elevated to prevent gravity-related intracranial pressure increases, possibly leading to intracranial bleeding. All areas of petechiae and ecchymoses are protected from further injury. Rest periods are provided between activities if the patient tires easily. Both patient and family are encouraged to discuss their concerns about the disease and its treatment, and emotional support is provided and questions answered honestly. The nurse reassures the patient that areas of petechiae and ecchymoses will heal as the disease resolves. The patient should avoid taking aspirin in any form as well as any other drugs that impair coagulation, including nonsteroidal anti-inflammatory drugs. If the patient experiences frequent nosebleeds, the patient should use a humidifier at night and should moisten the nostrils twice a day with saline. The nurse teaches the patient to monitor the condition by examining the skin for petechiae and ecchymoses and demonstrates the correct method to test stools for occult blood. If the patient is receiving corticosteroid therapy, fluid and electrolyte balance is monitored and the patient is assessed for signs of infection, pathological fractures, and mood changes. If the patient is receiving blood or blood components, they are administered according to protocol; vital signs are monitored before, during, and after the transfusion, and the patient is observed closely for adverse reactions. If the patient is receiving immunosuppressants, the patient is monitored closely for signs of bone marrow depression, opportunistic infections, mucositis, GI tract ulceration, and severe diarrhea or vomiting. If the patient is scheduled for a splenectomy, the nurse determines the patient's understanding of the procedure, corrects misinformation, administers prescribed blood transfusions, explains postoperative care and expected activities and sensations, ensures that a signed informed consent has been obtained, and prepares the patient physically (according to institutional or surgeon's protocol) and emotionally for the surgery. Postoperatively, all general patient care concerns apply. Normally, platelets increase spontaneously after splenectomy, but the patient may need initial postoperative support with blood and component replacement and platelet concentrate. The patient with chronic ITP should wear or carry a medical identification device.
Idiopathic Thrombocytopenic Purpura
|Mean LOS:||5 days|
|Description:||MEDICAL: Coagulation Disorders|
Idiopathic thrombocytopenic purpura (ITP) is an acquired hemorrhagic disorder characterized by an increased destruction of platelets because of antiplatelet antibodies. The antibodies attach to the platelets, reduce their life span, and lead to a platelet count below 100,000/mm3 and occasionally as low as 5,000/mm3. ITP can be divided into two categories: acute and chronic. Acute ITP is generally a self-limiting childhood disorder, whereas chronic ITP predominantly affects adults and is characterized by thrombocytopenia of more than 6 months.
The most life-threatening complication of ITP is intracerebral hemorrhage, which is most likely to occur if the platelet count falls below 1,000/mm3. Hemorrhage into the kidneys, abdominal cavity, or retroperitoneal space is also possible. Prognosis for acute ITP is excellent, with nearly 80% of patients recovering without treatment. The mortality rate from hemorrhage is 1% in children and 5% in adults. Older age and a previous history of hemorrhage increase the risk of severe bleeding in adults. Prognosis for chronic ITP is good, with remissions lasting weeks or even years.
Acute ITP is thought to be a response to a viral infection. Generally, a viral infection, such as rubella or chickenpox, occurs 2 to 21 days before the onset of the disease. Acute ITP may occur after live vaccine immunizations and is most prevalent during the winter and spring months when the incidence of infection is high. It is also associated with HIV. Chronic ITP generally has no underlying viral association and is often linked to immunological disorders, such as lupus erythematosus, or to drug reactions.
While a clear genetic contribution has not been established, ITP has been seen in monozygotic twins, and a predisposition to autoantibody production can run in families.
Gender, ethnic/racial, and life span considerations
Acute ITP affects children of both sexes and is most common between ages 2 and 4. About half of the children recover in 1 month, and 93% recover completely by 1 year. More than 80% of acute ITP patients recover, regardless of treatment, but 10% to 20% progress to chronic ITP. ITP can also occur during pregnancy, and 5% to 20% of the neonates born to these mothers will have severe thrombocytopenia and are at risk of intracranial hemorrhage during vaginal birth. Chronic ITP occurs mainly between the ages of 20 and 50 years and affects women almost three times as often as men. There are no known racial and ethnic considerations.
Global health considerations
Global incidence of ITP varies greatly depending on the country and ranges from 10 to 125 cases per 1 million per year in developed regions of the world such as North America, Western Europe, and the Middle East. Few data are available in developing regions.
Ask if the patient has recently had rubella or chickenpox or a viral infection with symptoms such as upper respiratory or gastrointestinal (GI). Ask if the patient was recently immunized with a live vaccine. Check for a history of systemic lupus erythematosus; easy bruising; or bleeding from the nose, gums, or GI or urinary tract. Because the symptoms of chronic ITP are usually insidious, patients may not have noticed an increase in symptoms. With a female patient, ask for the date of the last menstrual period, whether recent menses lasted longer and were heavier than usual, or whether she is pregnant. Ask if the patient has had HIV testing.
Physical examination of patients with acute ITP reveals diffuse petechiae (red to purple dots on the skin 1 to 3 mm in size) or bruises on the skin and in the oral mucosa. Chronic ITP patients may have no obvious petechiae. Other clinical features of ITP include ecchymoses (areas of purple to purplish-blue fading to green, yellow, and brown with time), which can occur anywhere on the body from even minor trauma. In both types of ITP, the spleen and liver are often slightly palpable with lymph node swelling. Ongoing assessment throughout patient management is essential to evaluate for signs of life-threatening bleeding.
Children with acute ITP are usually brought to the pediatrician by highly anxious parents who are concerned with the sudden appearance of easy bruising, petechiae, and occasionally bleeding gums and nosebleeds. Because these symptoms are so commonly associated with leukemia, parents and children need swift diagnosis and reassurance. Pregnant women are concerned about their own health as well as the health of the fetus.
|Test||Normal Result||Abnormality With Condition||Explanation|
|Platelet count||190,000–405,000/mm3||> 100,000/mm3||Platelets are consumed during clot formation; degree of platelet suppression predicts the severity of symptoms: 30,000–50,000/mm3, bruising with minor trauma; 15,000–30,000/mm3, spontaneous bruising, petechiae, particularly on the arms and legs; > 15,000/mm3, spontaneous bruising, mucosal bleeding, nosebleeds, bloody urine or stool, intracranial bleeding|
Other Tests: Tests include complete blood count and coagulation profiles, blood smear studies, bone marrow aspiration, HIV testing, and computed tomography of the head if intracerebral hemorrhage is suspected.
Primary nursing diagnosis
DiagnosisRisk of injury related to increased bleeding tendency
OutcomesRisk control; Safety behavior: Fall prevention; Knowledge: Personal safety; Safety status: Physical injury; Knowledge: Medication; Safety behavior: Home physical environment
InterventionsBleeding precautions; Bleeding reduction; Fall prevention; Environmental management; Safety; Health education; Surveillance; Medication management
Planning and implementation
Treatment for ITP is primarily pharmacologic. Because the risk of hemorrhage occurs early in the course of acute ITP, therapy is focused on a rapid, sustained elevation in platelet counts. Children with non-life-threatening bleeding are not generally given transfused platelets because the antiplatelet antibody found in their serum is directed against both autologous and transfused platelets.
If the patient fails to respond within 1 to 4 months or needs a high steroid dosage, splenectomy is usually considered. Splenectomy is effective because the spleen is a major site of antibody production and platelet destruction; research suggests that splenectomy is successful 85% of the time. In the face of life-threatening bleeding, such as intracranial or massive GI hemorrhages, a splenectomy is indicated.
|Medication or Drug Class||Dosage||Description||Rationale|
|Intravenous immune globulin; IV anti-(Rh)D (IV RhIG)||1 g/kg IV qd for 2 days||Immune serum||Increases antibody titer and antigen-antibody reaction; provides passive immunity against infection and induces rapid but short-term increases in platelet count|
|Glucocorticoids||Varies with drug||Prednisone 1–2 mg/kg PO qd; methylprednisolone 1–2 g IV for 3 days||Decrease inflammatory response; glucocorticoids are highly controversial therapy for children. Chronic ITP requires a slow steroid taper over several months|
Other Therapy: Alternative treatments include immunosuppression agents such as cyclophosphamide (Cytoxan) and vincristine sulfate. Plasmapheresis has been attempted with limited success.
Many children are managed as outpatients with frequent outpatient visits for therapeutics and platelet counts. If the platelet count is less than 15,000/mm3, the condition may be considered serious enough to warrant hospitalization. Institute safety precautions to prevent injury and the resultant bleeding and to assist with ambulation. Protect areas of hematoma, petechiae, and ecchymoses from further injury. Avoid intramuscular injections, but if they are essential, apply pressure for at least 10 minutes after the intramuscular injection and for 20 minutes after venipuncture. Avoid nasotracheal suctioning, if possible, to prevent bleeding. If a child is being managed as an outpatient, discuss the home environment with the parents or caregivers. Encourage the parents to set up one or two rooms at home (e.g., the child’s bedroom and the family room) as a protected environment. Pad all hard surfaces and corners with pillows and blankets and remove obstructions, furniture, and loose rugs.
Teach the patient and significant others about the nature of this disorder and necessary self-assessments and self-care activities. Teach the patient to report any signs of petechiae and ecchymoses formation, bruising, bleeding gums, and other signs of frank bleeding. Encourage the patient to stand unclothed in front of a mirror once a day to check for areas of bruising. Headaches and any change in level of consciousness may indicate cerebral bleeding and need to be reported to the healthcare worker immediately. Teach the signs and symptoms of blood loss, such as pallor or fatigue. Demonstrate correct mouth care for the patient and significant others by using a soft toothbrush to avoid mouth injury. Recommend electric shavers for both men and women. Teach the patient to use care when taking a rectal temperature to prevent rectal perforation. Recommend care when clipping fingernails or toenails. If any bleeding does occur, instruct the patient to apply pressure to the area for up to 15 minutes or to seek help. Teach the patient to avoid aspirin, ibuprofen in any form, and other drugs that impair coagulation, with particular attention to over-the-counter remedies.
Provide a private, quiet environment to discuss the patient’s or parents’ concerns. The period of diagnosis is an anxious one, and parents need a great deal of emotional support. If the child is managed at home, parents need an opportunity to express their fears.
Evidence-Based Practice and Health Policy
Bansal, D., Bhamare, T.A., Trehan, A., Ahluwalia, J., Varma, N., & Marwaha, R.K. (2010). Outcome of chronic idiopathic thrombocytopenic purpura in children. Pediatric Blood and Cancer, 54(3), 403–407.
- Investigators reviewed the outcomes for 270 children diagnosed with ITP over a period of 30 months (range, 6 to 166 months). The median diagnosis age was 6 years (range, 1 to 14 years).
- In this sample, 37.7% were diagnosed with acute ITP, 49.6% were diagnosed with chronic ITP, and 12.7% were unknown. More than half of the children (50.7%) had persistent thrombocytopenia.
- Seventy-six percent of the children received some form of pharmacologic therapy, and 18% received a splenectomy. Half of the sample had no response to therapy, and 24.8% experienced complete remission. The remaining children experienced either relapse (10.7%) or partial remission (13.7%).
- The probability of remission at 5 years was 24% for males and 39.6% for females (p = 0.01). At 10 years, the probability of remission was 34% for males and 50.6% for females (p = 0.01).
- Physical findings of skin and mucous membranes: Presence of petechiae, ecchymoses, blood blisters, hematoma, bleeding
- Reaction to rest and activity
- Presence of complications: Bleeding, petechiae, ecchymoses, headache, increased bruising
Discharge and home healthcare guidelines
To prevent bleeding episodes, the patient should avoid both physical activity that may lead to injury and medications that have anticoagulant properties. Instruct the patient or caregiver when to notify the physician and how to monitor for bleeding in the stool, urine, and sputum. Remind the patient or caregiver to notify any medical personnel of bleeding tendencies. If the patient is a school-age child, encourage the parents to notify the school of the diagnosis, treatment, and complications.
Explain all discharge medications, including dosage, route, action, adverse effects, and need for routine laboratory monitoring. If the patient is being discharged on a tapering corticosteroid dosage, be sure the patient or caregiver understands the schedule. If the patient had a central line placed for intravenous therapy, be sure the patient or caregiver has been properly trained in care, dressing changes, and sterile techniques. Teach the patient that antacids and oral drugs taken with meals can reduce gastric irritation. Weight gain, anxiety, and mood alterations are frequent side effects of steroid therapy. Parents and families need to be encouraged to lift activity restrictions when the child’s platelet count returns to a safe range.