idiopathic thrombocytopenic purpura


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Idiopathic Thrombocytopenic Purpura

 

Definition

Idiopathic thrombocytopenic purpura, or ITP, is a bleeding disorder caused by an abnormally low level of platelets in the patient's blood. Platelets are small plate-shaped bodies in the blood that combine to form a plug when a blood vessel is injured. The platelet plug then binds certain proteins in the blood to form a clot that stops bleeding. ITP's name describes its cause and two symptoms. Idiopathic means that the disorder has no apparent cause. ITP is now often called immune thrombocytopenic purpura rather than idiopathic because of recent findings that ITP patients have autoimmune antibodies in their blood. Thrombocytopenia is another word for a decreased number of blood platelets. Purpura refers to a purplish or reddish-brown skin rash caused by the leakage of blood from broken capillaries into the skin. Other names for ITP include purpura hemorrhagica and essential thrombocytopenia.

Description

ITP may be either acute or chronic. The acute form is most common in children between the ages of two and six years; the chronic form is most common in adult females between 20 and 40. Between 10% and 20% of children with ITP have the chronic form. ITP does not appear to be related to race, lifestyle, climate, or environmental factors.
ITP is a disorder that affects the overall number of blood platelets rather than their function. The normal platelet level in adults is between 150,000 and 450,000/mm3. Platelet counts below 50,000 mm3 increase the risk of dangerous bleeding from trauma; counts below 20,000/mm3 increase the risk of spontaneous bleeding.

Causes and symptoms

In adults, ITP is considered an autoimmune disorder, which means that the body produces antibodies that damage some of its own products—in this case, blood platelets. Some adults with chronic ITP also have other immune system disorders, such as systemic lupus erythematosus (SLE). In children, ITP is usually triggered by a virus infection, most often rubella, chickenpox, measles, cytomegalovirus, or Epstein-Barr virus. It usually begins about two or three weeks after the infection.

Acute itp

Acute ITP is characterized by bleeding into the skin or from the nose, mouth, digestive tract, or urinary tract. The onset is usually sudden. Bleeding into the skin takes the form of purpura or petechiae. Purpura is a purplish or reddish-brown rash or discoloration of the skin; petechiae are small round pinpoint hemorrhages. Both are caused by the leakage of blood from tiny capillaries under the skin surface. In addition to purpura and petechiae, the patient may notice that he or she bruises more easily than usual. In extreme cases, patients with ITP may bleed into the lungs, brain, or other vital organs.

Chronic itp

Chronic ITP has a gradual onset and may have minimal or no external symptoms. The low platelet count may be discovered in the course of a routine blood test. Most patients with chronic ITP, however, will consult their primary care doctor because of the purpuric skin rash, nosebleeds, or bleeding from the digestive or urinary tract. Women sometimes go to their gynecologist for unusually heavy or lengthy menstrual periods.
The risk factors for the development of chronic ITP include:
  • female sex
  • age over 10 years at onset of symptoms
  • slow onset of bruising
  • presence of other autoantibodies in the blood

Diagnosis

ITP is usually considered a diagnosis of exclusion, which means that the doctor arrives at the diagnosis by a process of ruling out other possible causes. If the patient belongs to one or more of the risk groups for chronic ITP, the doctor may order a blood test for autoantibodies in the blood early in the diagnostic process.

Physical examination

If the doctor suspects ITP, he or she will examine the patient's skin for bruises, purpuric areas, or petechiae. If the patient has had nosebleeds or bleeding from the mouth or other parts of the body, the doctor will examine these areas for other possible causes of bleeding.
Patients with ITP usually look and feel healthy except for the bleeding.
The most important features that the doctor will be looking for during the physical examination are the condition of the patient's spleen and the presence of fever. Patients with ITP do not have fever, whereas patients with lupus and some other types of thrombocytopenia are usually feverish. The doctor will have the patient lie flat on the examining table in order to feel the size of the spleen. If the spleen is noticeably enlarged, ITP is not absolutely ruled out but is a less likely diagnosis.

Laboratory testing

The doctor will order a complete blood count (CBC), a test of clotting time, a bone marrow test, and a test for antiplatelet antibodies if it is available in the hospital laboratory. Patients with ITP usually have platelet counts below 20,000/mm3 and prolonged bleeding time. The size and appearance of the platelets may be abnormal. The red blood cell count (RBC) and white blood cell count (WBC) are usually normal, although about 10% of patients with ITP are also anemic. The blood marrow test yields normal results. Detection of antiplatelet antibodies in the blood is considered to confirm the diagnosis of ITP.

Treatment

General care and monitoring

There is no specific treatment for ITP. In most cases, the disorder will resolve without medications or surgery within two to six weeks. Nosebleeds can be treated with ice packs when necessary.
General care includes explaining ITP to the patient and advising him or her to watch for bruising, petechiae, or other signs of recurrence. Children should be discouraged from rough contact sports or other activities that increase the risk of trauma. Patients are also advised to avoid using aspirin or ibuprofen (Advil, Motrin) as pain relievers because these drugs lengthen the clotting time of blood.

Emergency treatment

Patients with acute ITP who are losing large amounts of blood or bleeding into their central nervous system require emergency treatment. This includes transfusions of platelets, intravenous immunoglobulins, or prednisone. Prednisone is a steroid medication that decreases the effects of antibody on platelets and eventually lowers antibody production. If the patient has a history of ITP that has not responded to prednisone or immunoglobulins, the surgeon may remove the patient's spleen. This operation is called a splenectomy. The reason for removing the spleen when ITP does not respond to other forms of treatment is that the spleen sometimes keeps platelets out of the general blood circulation.

Medications and transfusions

Patients with chronic ITP can be treated with prednisone, immune globulin, or large doses of intravenous gamma globulin. Although 90% of patients respond to immunoglobulin treatment, it is very expensive. About 80% of patients respond to prednisone therapy. Platelet transfusions are not recommended for routine treatment of ITP. If the patient's platelet level does not improve within one to four months, or requires high doses of prednisone, the doctor may recommend splenectomy. All medications for ITP are given either orally or intravenously; intramuscular injection is avoided because of the possibility of causing bleeding into the skin.

Surgery

Between 80% and 85% of adults with ITP have a remission of the disorder after the spleen is removed. Splenectomy is usually avoided in children younger than five years because of the increased risk of a severe infection after the operation. In older children, however, splenectomy is recommended if the child has been treated for 12 months without improvement; if the ITP is very severe or the patient is getting worse; if the patient begins to bleed into the head or brain; and if the patient is an adolescent female with extremely heavy periods.

Prognosis

The prognosis for recovery from acute ITP is good; 80% of patients recover without special treatment. The prognosis for chronic ITP is also good; most patients experience long-term remissions. In rare instances, however, ITP can cause life-threatening hemorrhage or bleeding into the central nervous system.

Resources

Books

Linker, Charles A. "Blood." In Current Medical Diagnosis and Treatment, 1998, edited by Stephen McPhee, et al., 37th ed. Stamford: Appleton & Lange, 1997.

Key terms

Autoimmune disorder — A disorder in which the patient's immune system produces antibodies that destroy some of the body's own products. ITP in adults is thought to be an autoimmune disorder.
Idiopathic — Of unknown cause. Idiopathic refers to a disease that is not preceded or caused by any known dysfunction or disorder in the body.
Petechiae — Small pinpoint hemorrhages in skin or mucous membranes caused by the rupture of capillaries.
Platelet — A blood component that helps to prevent blood from leaking from broken blood vessels. ITP is a bleeding disorder caused by an abnormally low level of platelets in the blood.
Prednisone — A corticosteroid medication that is used to treat ITP. Prednisone works by decreasing the effects of antibody on blood platelets. Longterm treatment with prednisone is thought to decrease antibody production.
Purpura — A skin discoloration of purplish or brownish red spots caused by bleeding from broken capillaries.
Splenectomy — Surgical removal of the spleen.
Thrombocytopenia — An abnormal decline in the number of platelets in the blood.

purpura

 [per´pu-rah]
a hemorrhagic disease characterized by extravasation of blood into the tissues, under the skin, and through the mucous membranes, and producing spontaneous bruises, ecchymoses, and petechiae (small hemorrhagic spots) on the skin. (See plate in Dermatology Atlas.) When accompanied by a decrease in the circulating platelets, it is called thrombocytopenic purpura; when there is no decrease in the platelet count, it is called nonthrombocytopenic purpura. adj., adj purpu´ric.

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).
Symptoms. The outward manifestations and laboratory findings of primary and secondary thrombocytopenic purpura are similar. There is evidence of bleeding under the skin, with easy bruising and the development of petechiae. In the acute form there may be bleeding from any of the body orifices, such as hematuria, nosebleed, vaginal bleeding, and bleeding gums. The platelet count is below 100,000 per cubic millimeter of blood and may go as low as 10,000 per cubic millimeter (normal count is about 250,000 per cubic millimeter). The bleeding time is prolonged and clot retraction is poor. Coagulation time is normal.
Treatment. Differential diagnosis is necessary to determine the type of purpura present and to eliminate the cause if it can be determined. General measures include protection of the patient from trauma, elective surgery, and tooth extractions, any one of which may lead to severe or even fatal hemorrhage. In the thrombocytopenic form, corticosteroids may be administered when the purpura is moderately severe and of short duration. Splenectomy is indicated when other, more conservative measures fail and is successful in a majority of cases. In some instances, especially in children, there may be spontaneous and permanent recovery from idiopathic purpura. (See Atlas 2, Part Q.)
allergic purpura (anaphylactoid purpura) Henoch-Schönlein purpura.
annular telangiectatic purpura a rare form in which pinpoint reddened lesions coalesce to form a ring-shaped or continuously linked pattern, commonly beginning in the lower extremities and becoming generalized. Called also Majocchi's disease.
fibrinolytic purpura purpura associated with increased fibrinolytic activity of the blood.
purpura ful´minans a form of nonthrombocytopenic purpura seen mainly in children, usually after an infectious disease, marked by fever, shock, anemia, and sudden, rapidly spreading symmetrical skin hemorrhages of the lower limbs, often associated with extensive intravascular thromboses and gangrene.
purpura hemorrha´gica primary thrombocytopenic purpura.
Henoch's purpura a type of Henoch-Schönlein purpura in which abdominal symptoms predominate.
Henoch-Schönlein purpura a type of nonthrombocytopenic purpura, of unknown cause but thought to be due to a vasculitis; it is most often seen in children and is associated with clinical symptoms such as urticaria and erythema, arthritis and other joint diseases, gastrointestinal symptoms, and renal involvement. Called also Schönlein-Henoch purpura or syndrome and allergic or anaphylactoid purpura.
idiopathic thrombocytopenic purpura thrombocytopenic purpura not directly associated with any definable systemic disease, although it often follows a systemic infection; the cause is thought to be an IgG immunoglobulin that acts as an antibody against platelets, causing ecchymoses, petechiae, and other bleeding. There are both acute and chronic forms: the acute form has a sudden onset, is more common in children, and usually resolves spontaneously within a few months; the chronic form has a slower onset, is more common in adults, and may be recurrent.
nonthrombocytopenic purpura purpura without any decrease in the platelet count of the blood. In such cases the cause is either abnormal capillary fragility or a clotting factor deficiency.
Schönlein purpura a type of Henoch-Schönlein purpura in which articular systems predominate; called also Schönlein's disease.
Schönlein-Henoch purpura Henoch-Schönlein purpura.
purpura seni´lis dark purplish red ecchymoses occurring on the forearms and backs of the hands in the elderly.
thrombocytopenic purpura purpura associated with a decrease in the number of platelets in the blood; see purpura.
thrombotic thrombocytopenic purpura a form of thrombotic microangiopathy characterized by thrombocytopenia, hemolytic anemia, bizarre neurological manifestations, uremia (azotemia), fever, and thromboses in terminal arterioles and capillaries; some authorities consider it identical to the hemolytic uremic syndrome. Called also microangiopathic hemolytic anemia and Moschcowitz's disease.

id·i·o·path·ic throm·bo·cy·to·pe·nic pur·pu·ra (ITP),

a systemic illness characterized by extensive ecchymoses and hemorrhages from mucous membranes and very low platelet counts; resulting from platelet destruction by macrophages due to an antiplatelet factor; childhood cases are usually brief and rarely present with intracranial hemorrhages, but adult cases are often recurrent and have a higher incidence of grave bleeding, especially intracranial.

idiopathic thrombocytopenic purpura (ITP),

a deficiency of platelets that results in bleeding into the skin and other organs. Acute ITP is a disease of children that may follow a viral infection, lasts a few weeks to a few months, and usually has no residual effects. Chronic ITP is more common in adolescents and adults, begins more insidiously, and lasts longer. Antibodies to platelets are found in patients with ITP. The condition may be transmitted to the fetus if the mother is affected. Treatment includes hemophoresis, corticosteroids, therapeutic plasmapheresis, and splenectomy. See also thrombocytopenia, thrombocytopenic purpura.

idiopathic thrombocytopenic purpura

Werlhof'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;23 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
ITP clinical features   Acute Chronic
Peak age Children, age 2-6 Adults, age 20-40
Sex (♀:♂ratio) 1:1 3:1
History, recent infection Common Rare
Onset  Abrupt Insidious
Hemorrhagic bullae-oral Present if severe Rare
Eosinophilia, lymphocytosis Common Rare
Platelet count < 20 x 109/L 30-80 x 109/L
Duration 2-6 weeks Months, years
Spontaneous remission Common, ± 80% Rare, ± 20%  
After TC Bithell, in GR Lee, et al Eds, Wintrobe's Clinical Hematology, 9th ed, Lea & Febiger, Philadelphia, 1993

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)
A systemic illness characterized by extensive ecchymoses and hemorrhages from mucous membranes and very low platelet counts; resulting from destruction in the spleen of platelets to which an autoimmune globulin is bound; childhood cases, which often follow viral infection, are mild and transitory; in adults, bleeding may be recurrent and severe.
Synonym(s): immune thrombocytopenic purpura, purpura hemorrhagica, thrombopenic purpura.
Enlarge picture
IDIOPATHIC THROMBOCYTOPENIC PURPURA: Virtual absence of platelets in peripheral blood (×400)

idiopathic thrombocytopenic purpura

Abbreviation: ITP
A hemorrhagic autoimmune disease in which there is destruction of circulating platelets, caused by antiplatelet autoantibodies that bind with antigens on the platelet membrane, making platelets more susceptible to phagocytosis and destruction in the spleen. It occurs as an acute disease in children, usually between ages 2 and 6, and often follows a viral infection. Chronic ITP seldom follows an infection and is commonly linked to immunologic disorders such as lupus erythematosis or patients with acquired immunodeficiency syndrome who are exposed to the rubella virus. It also is linked to drug reactions, and occurs in cases of alcohol, heroin, or morphine abuse. It mainly affects adults younger than age 50, especially women between 20 and 40. Opsonization of platelets by autoantibodies stimulates their lysis by macrophages, esp. in the spleen. Synonym: Henoch-Schönlein disease; hemorrhagic purpura; thrombocytopenic purpura; thrombopenic purpuraillustration;

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

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.

Treatment

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.

Patient care

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.

See also: purpura

Idiopathic Thrombocytopenic Purpura

DRG Category:813
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.

Causes

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.

Genetic considerations

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.

Assessment

History

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

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.

Psychosocial

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.

Diagnostic highlights

TestNormal ResultAbnormality With ConditionExplanation
Platelet count190,000–405,000/mm3> 100,000/mm3Platelets 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

Diagnosis

Risk of injury related to increased bleeding tendency

Outcomes

Risk control; Safety behavior: Fall prevention; Knowledge: Personal safety; Safety status: Physical injury; Knowledge: Medication; Safety behavior: Home physical environment

Interventions

Bleeding precautions; Bleeding reduction; Fall prevention; Environmental management; Safety; Health education; Surveillance; Medication management

Planning and implementation

Collaborative

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.

Pharmacologic highlights

Medication or Drug ClassDosageDescriptionRationale
Intravenous immune globulin; IV anti-(Rh)D (IV RhIG)1 g/kg IV qd for 2 daysImmune serumIncreases antibody titer and antigen-antibody reaction; provides passive immunity against infection and induces rapid but short-term increases in platelet count
GlucocorticoidsVaries with drugPrednisone 1–2 mg/kg PO qd; methylprednisolone 1–2 g IV for 3 daysDecrease 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.

Independent

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).

Documentation guidelines

  • 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.

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)
Systemic illness characterized by extensive ecchymoses and hemorrhages from mucous membranes and very low platelet counts; due to platelet destruction by macrophages due to an antiplatelet factor.
References in periodicals archive ?
influenzae) pneumonia, with associated idiopathic thrombocytopenic purpura (ITP) and autonomic perturbation manifesting as severe hyperthermia.
InKine's portfolio also includes two late-stage product candidates, Hematrol(TM) in Phase III trials for the treatment of idiopathic thrombocytopenic purpura, and Colirest(TM) in Phase II trials for the treatment of inflammatory bowel disease.
InKine's portfolio also includes a late-stage clinical compound, CBP-1011, a steroid molecule in Phase III trials for the treatment of idiopathic thrombocytopenic purpura and Phase II trials for the treatment of inflammatory bowel disease.
is a biotechnology company engaged in the development of a new class of drugs for the treatment of Rheumatoid Arthritis (RA), Idiopathic Thrombocytopenic Purpura (ITP), and a host of other autoimmune disorders.
Current therapy with immunosuppressive drugs often has severe side effects and our data to date using CBP-1011 in Crohn's and idiopathic thrombocytopenic purpura (ITP) supports a safety benefit.
is a biotechnology company engaged in the development of a new class of drugs for the treatment of rheumatoid arthritis, idiopathic thrombocytopenic purpura and a host of other autoimmune disorders.
Analysis of data from this study showed that Promacta at 50mg or 75mg resulted in a positive trend in decrease in bleeding incidence (all grades) in adult patients with chronic idiopathic thrombocytopenic purpura (ITP), an autoimmune condition which results in low blood platelet counts and bleeding from the small blood vessels.
com/research/9d6811/thrombocytopenia_a) has announced the addition of the "Thrombocytopenia and Idiopathic Thrombocytopenic Purpura Therapy Area Pipeline Report" report to their offering.
Data from this study showed that PROMACTA at 50-75mg once daily resulted in a statistically significant increase in platelet counts and also reduced bleeding in adult patients with chronic idiopathic thrombocytopenic purpura (ITP).

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