IDIOPATHIC THROMBOCYTOPENIC PURPURA (ITP)

Idiopathic thrombocytopenic purpura (ITP) may occur when the immune system mistakenly attacks platelets.

1. IDIOPATHIC THROMBOCYTOPENIC PURPURA (ITP) P resented By; jagdish sambad MSC nursing IKDRC college of nursing

2. What is ITP ? I- idiopathic T-thrombocytopenic P-purpura

3.  Definition : Idiopathic thrombocytopenic purpura is a blood disorder characterized by an abnormal decrease in the number of platelets in the blood , can result in easy bruising, bleeding gums and internal bleeding.  "Idiopathic" means the cause is unknown.  "Thrombocytopenia" means a decreased number of platelets in the blood.  "Purpura" refers to the purple discoloring of the skin, as with a bruise.

4.  There are two forms of ITP:  Acute thrombocytopenic purpura — This is most commonly seen in young children (2 to 6 years old). The symptoms may follow a viral illness, such as chickenpox. Acute ITP usually has a very sudden onset and the symptoms usually disappear in less than six months (often within a few weeks). The disorder usually does not recur. Acute ITP is the most common form of the disorder.  Chronic thrombocytopenic purpura — The onset of the disorder can happen at any age, and the symptoms can last a minimum of six months to several years. Adults have this form more often than children, but it does affect adolescents. Chronic ITP can recur often and requires continual follow-up care with a blood specialist .

5.  Idiopathic thrombocytopenic purpura ( ITP ),  immune thrombocytopenia ,  primary immune thrombocytopenia,  primary immune thrombocytopenic purpura  autoimmune thrombocytopenic purpura

6. C auses of ITP: Inherited: fanconi syndrome, hereditary thrombocytopenia Immune: ITP, neonatal alloimmune thrombocytopenia Non-immune: I) Shortened circulation Thrombotic thrombocytopenic purpura (TTIP) Disseminated intravascular coagulation (DIC) Heparin induced thrombocytopenia (HIT) Splenomegaly II) Decreased circulation Drug induced marrow suppression Chemotherapy Viral infection Bacterial infection Alcoholism/ bone marrow suppression, MDS, myelofibrosis, aplastic anemia, radiation to bone.

7. pathophysiology  In approximately 60 percent of cases, antibodies against platelets can be detected.  Most often these are membrane glycoproteins IIb-IIIa or Ib-IX , and are of the (IgG) type.  The coating of platelets with IgG renders them susceptible to opsonization and phagocytosis by splenic macrophages, as well by Kupffer cells in the liver  . The IgG autoantibodies are also thought to damage megakaryocytes, the precursor cells to platelets,  Recent research now indicates that impaired production of the hormone thrombopoietin, which is the stimulant for platelet production, may be a contributing factor to the reduction in circulating platelets.  The stimulus for auto-antibody production in ITP is probably abnormal T cell activity. Preliminary findings suggest that these T cells can be influenced by drugs that target B cells, such as rituximab .

8. Glanzmann's thrombasthenia is an abnormality of the platelets . It is an extremely rare coagulopathy (bleeding disorder due to a blood abnormality), in which the platelets contain defective or low levels of glycoprotein IIb / IIIa ( GpIIb / IIIa ) , which is a receptor for fibrinogen . As a result, no fibrinogen bridging of platelets to other platelets can occur, and the bleeding time is significantly prolonged. Bernard– Soulier syndrome (BSS), also called hemorrhagiparous thrombocytic dystrophy ,is a rare autosomal recessive bleeding disorder that causes a deficiency of glycoprotein Ib ( GpIb ), the receptor for von Willebrand factor . The incidence of BSS is estimated to be less than 1 case per million persons, based on cases reported from Europe, North America, and Japan. BSS is a giant platelet disorder , meaning that it is characterized by abnormally large platelets. Von Willebrand disease ( vWD ) is the most common hereditary blood-clotting disorder in humans. An acquired form can sometimes result from other medical conditions. [1] It arises from a deficiency in the quality or quantity of von Willebrand factor ( vWF ), a multimeric protein that is required for platelet adhesion . As well as humans, it is known to affect several breeds of dogs.

9. In most cases, the cause of ITP is unknown and It is not contagious.  Often, the child may have had a virus or viral infection about three weeks before developing ITP. It is believed that the body, when making antibodies against a virus, "accidentally" also made an antibody that can stick to the platelet cells. The body recognizes any cells with antibodies as foreign cells and destroys them. That is why ITP is also referred to as immune thrombocytopenic purpura.

10.  The bone marrow responds to the low number of platelets and produces many more to send out to the body. in the bone marrow many young platelets that have been produced. However, the blood test results of the circulating blood would show a very low number of platelets.  The body is producing the cells normally, but the body is also destroying them. In most cases, other blood tests are normal except for the low number of platelets. ITP platelets usually survive only a few hours, in comparison to normal platelets which have a lifespan of 7 to 10 days.  Platelets are essential for the formation of a blood clot. Blood clots consist of a mass of fibers and blood cells. Platelets travel to a damaged area and stick together to form a plug, whenever a person is cut, for example. If there are not enough platelets, a clot cannot be formed, resulting in more bleeding.

11.  Signs and symptoms  Normal platelet count is in the range of 150,000 to 450,000. In a child with ITP, the platelet count is generally less than 100,000. By the time significant bleeding occurs, the child may have a platelet count of less than 10,000. The lower the platelet count, the greater the risk of bleeding.  Because platelets help stop bleeding, the symptoms of ITP are related to increased bleeding.  Purpura - the purple color of the skin after blood has "leaked" under it. A bruise is blood under the skin. Children with ITP may have large bruises from no known trauma. Bruises can appear at the joints of elbows and knees just from movement.  Petechia - tiny red dots under the skin that are a result of very small bleeds.  Nose bleeds  Bleeding in the mouth and/or in and around the gums  Blood in the vomit, urine or stool  Bleeding in the head - this is the most dangerous symptom of ITP. Any head trauma that occurs when there are not enough platelets to stop the bleeding can be life threatening.

12.  Testing and diagnosis  The diagnosis of ITP is a process of exclusion.  First, it has to be determined that there are no blood abnormalities other than a low platelet count, and no physical signs other than bleeding. Then, secondary causes (5– 10 percent of suspected ITP cases) should be excluded.  secondary causes include leukemia ,  medications (e.g., quinine , heparin ),  lupus erythematosus ,  cirrhosis ,  HIV , hepatitis C, congenital causes, antiphospholipid syndrome , von Willebrand factor deficiency,  Despite the destruction of platelets by splenic macrophages, the spleen is normally not enlarged.  In fact, an enlarged spleen should lead to a search for other possible causes for the thrombocytopenia.  Bleeding time is usually prolonged in ITP patients. a normal bleeding time does not exclude a platelet disorder.

13.  Bone marrow examination may be performed on patients over the age of 60 and those who do not respond to treatment, or when the diagnosis is in doubt.  On examination of the marrow, an increase in the production of megakaryocytes may be observed and may help in establishing a diagnosis  A complete blood count (CBC) , which measures the size, number, and maturity of different blood cells  Additional blood and urine tests, which measure bleeding time and detect possible infections  Careful review of the child's medications  A bone marrow aspiration may be performed to look at child's production of platelets and to rule out any abnormal cells your child's bone marrow could be producing that could lower platelet counts.

14. Treatment Corticosteroids Platelet transfusion Splenectomy Intravenous immunoglobulin Anti-Rh D Danazol Immunosuppressive medicine High-dose cyclophosphamide or combination therapy

15.  Treatment-  Not all children with ITP require treatment. Many children spontaneously recover in about 2 to 4 day  Steroids, which help prevent bleeding by decreasing the rate of platelet destruction. Steroids, if effective, will result in an increase in platelet counts seen within two to three weeks. Initial treatment usually consists of the administration of corticosteroids , in urgent situations, infusions of dexamethasone or methylprednisolone may be used, while oral prednisone or prednisolone may suffice in less severe cases

16. Anti-D Another option, suitable for Rh-positive , non-splenectomized patients is intravenous administration of Rho(D) immune globulin [Human; Anti-D]. The mechanism of action of anti-D is not fully understood. However, following administration, anti-D-coated red blood cell complexes saturate Fcγ receptor sites on macrophages , resulting in preferential destruction of red blood cells (RBCs), therefore sparing antibody-coated platelets . Steroid-sparing agents Immunosuppresants such as mycophenolate mofetil and azathioprine vincristine , a chemotherapy agent,and vinca alkaloid , has significant side- effects and To be used with caution, especially in children. Intravenous immunoglobulin (IVIg) may be infused in some cases. it is costly and produces improvement that generally lasts less than a month.

17. Thrombopoietin receptor agonists - are pharmaceutical agents that stimulate platelet production in the bone marrow. Two such products are currently available: Romiplostim (trade name Nplate) is a thrombopoiesis stimulating Fc- peptide fusion protein that is administered by subcutaneous injection . romiplostim is effective in treating chronic ITP, especially in relapsed post-splenectomy patients. Eltrombopag (trade name Promacta ) is an orally-administered agent with an effect similar to that of romiplostim. It too has been demonstrated to increase platelet counts and decrease bleeding in a dose-dependent manner. Side effects of thrombopoietin receptor agonists include headache, joint or muscle pain, dizziness, nausea or vomiting, and an increased risk of blood clots.

18. Surgery Splenectomy may be considered, as platelets which have been bound by antibodies are taken up by macrophages in the spleen . The procedure is potentially risky in ITP cases due to the increased possibility of significant bleeding during surgery. Platelet transfusion Platelet transfusion alone is normally not recommended except in an emergency, and is usually unsuccessful in producing a long-term platelet count increase. This is because the underlying autoimmune mechanism that is destroying the patient's platelets will also destroy donor platelets. H. pylori eradication In adults, particularly those living in areas with a high prevalence , identification and treatment of this infection has been shown to improve platelet counts in a third of patients.

19. Experimental and novel agents Dapsone is an anti-infective sulfone drug. In recent years, Dapsone has also proved helpful in treating lupus, rheumatoid arthritis and used as second-line treatment for ITP. The exact mechanism by which Dapsone assists in ITP is unclear. The off-label use of rituximab , a chimeric monoclonal antibody against the B cell surface antigen CD20 , has been shown in preliminary studies to be an effective alternative to Splenectomy in some patients. Promising results have been reported with kinase inhibitor tamatinib fosdium

20. Nursing assessment Subjective data: Past health history; recent hemorrhage, excessive bleeding or viral infection, exposure to radiation. Medication; use of thiazide diuretic, furosemide, aspirin, NSAID,penicilin, sulphonamides, methyldopa, and phenytoin. Objective data: Fever, lethargy, petechiae, ecchymosis, purpura, splenomegaly, abdominal distention, platelet count < 1,50,000 ul, prolonged bleeding time, decrease hemoglobin and hematocrit.

21. Nursing diagnosis Impaired oral mucous membrane related to low platelet counts effect of pathologic condition and treatment as evidenced by oral bleeding and blood –filled bullae G oal: experiences lesion-free oral mucosa without bleeding Intervention: Monitor lip, tongue, mucous membrane, tonsillar fossae and gums for moisture. Assist the patient to select soft, bland and non acidic food to decrease irritation of oral mucosa. Use a soft toothbrush for removal of dental debris. Use of toothpastes or disposable foam swab to stimulate and clean cavity with minimal trauma to gingiva. Assist patient to perform oral hygiene after eating and as often as needed to avoid breakdown of oral mucosa. Avoid use of lemon- glycerin swabs to prevent excessive drying of the oral mucosa.

22. Nursing diagnosis 2. Risk for injury related to low platelet count and treatments. Goal: maintain tissue integrity Intervention: - Monitor for signs and symptoms of persistent bleeding. Monitor coagulation studies , including prothrombin time, partial thromboplastin time. Avoid injections to prevent bleeding. Protect patient from trauma that may cause bleeding to reduce tissue trauma and subsequent bleeding into tissue. Administer blood product to replace coagulation factor. Teach patient to avoid aspirin or other anticoagulant to prevent additional bleeding risk.

23. Nursing diagnosis 3. Ineffective management of therapeutic regimen related to lack of knowledge of disease process, activity and medication as evidenced by frequent questioning about disease management, anxiety and restlessness. Goal: verbalize required knowledge and skill to manage disease process at home. Intervention: Assess the patient current knowledge level related to specific disease process. Describe disease process. Describe common sign and symptoms of disease. Discuss treatment option to decrease anxiety level of patient. Refer to patient local community support groups or agencies.

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