Most adults with ITP will eventually need treatment, as the condition often becomes severe or long term (chronic). Treatment may include a number of approaches, such as medications to boost your platelet count or surgery to remove your spleen (splenectomy). Talk with your doctor about the risks and benefits of your treatment options.
How is thrombocytopenia treated in systemic lupus erythematosus (SLE)?
With thrombocytopenia, treatment should be considered if bleeding or severe bruising are present, or with platelet counts <50 × 10 9 /l. There are no randomized controlled trials of the use of corticosteroids in the treatment of peripheral blood cytopenias in SLE.
What is the treatment for persistent ITP thrombocytopenia?
Persistent ITP Thrombocytopenia recurs in most patients when corticosteroids are tapered. We treat patients with persistent ITP with the goal of maintaining the platelet count more than 20 000 to 30 000 109/L while avoiding steroid-induced osteoporosis, cataracts, and other toxicities.
What is the best immunoglobulin for ITP?
Intravenous immunoglobulins (IVIG) are well established in the treatment of ITP. They are very effective in some patients with lupus associated thrombocytopenia who show a long-lasting response to treatment [31,32].
How effective is IVIG in the treatment of lupus-Associated Thrombocytopenia?
IVIG can be very effective in some patients with lupus-associated thrombocytopenia. Its use in this context and for other manifestations of SLE has recently been reviewed. IVIG is well established as a treatment option in ITP, and a long-lasting response in lupus-associated thrombocytopenia has been reported.
How do I treat ITP blood?
Medications to treat ITP may include:Steroids. Your doctor will likely start you on an oral corticosteroid, such as prednisone. ... Immune globulin. If corticosteroids don't help, your doctor may give you an injection of immune globulin. ... Drugs that boost platelet production. ... Other drugs.
How is ITP 2020 treated?
Current treatment of ITP is not strictly regimented. First-line therapy usually consists of steroids (high-dose dexamethasone or prednisone) or IV immunoglobulin (IVIG), or even a combination of both for certain patients.
What is the latest treatment for ITP?
Newer treatments, such as the thrombopoietin receptor agonists, have transformed ITP care. They have high efficacy, are well tolerated and improve patients' quality of life. A greater understanding of the underlying pathophysiology of this disorder has helped develop a number of new targeted therapies.
What is first-line therapy for ITP?
The standard initial treatment for ITP is oral corticosteroids to increase platelet counts. Intravenous immunoglobulin or anti-D immunoglobulin can also increase platelet counts and are particularly useful for stimulating rapid platelet increases before planned procedures.
How is ITP 2021 treated?
In adults with newly diagnosed ITP, the ASH guideline panel recommends against a prolonged course (>6 weeks) of prednisone in favor of a short course (≤6 weeks) and suggests either prednisone (0.5 - 2.0 mg/kg/day) or dexamethasone (40 mg/day for 4 days) as the type of corticosteroid for initial therapy1 .
Is ITP curable?
A: While there is no cure for ITP, many patients find their platelet count improves following treatment. What proves difficult for many ITP patients is finding the treatment that works for them without unwanted side effects. Some patients report that changing their diet or lifestyle helps them feel better.
Is there any medicine to increase platelets?
Romiplostim injection is used to increase the number of platelets (cells that help the blood to clot) in order to decrease the risk of bleeding in adults who have immune thrombocytopenia (ITP; idiopathic thrombocytopenic purpura; an ongoing condition that may cause easy bruising or bleeding due to an abnormally low ...
How long should I take steroids for ITP?
In light of the heavy treatment burden of prolonged corticosteroid exposure, clinical practice guidelines recommend limiting corticosteroid treatment to no more than 6 weeks in adults with ITP receiving initial therapy.
How long does it take to cure ITP?
ITP may be acute and resolve in less than 6 months, or chronic and last longer than 6 months. Treatment options include a variety of medications that can reduce the destruction of platelets or increase their production. In some cases, surgery to remove the spleen is necessary.
Why are steroids given in ITP?
Corticosteroids ("steroids") — Steroids prevent bleeding by decreasing the production of antibodies against platelets. If effective, the platelet count will rise within two to four weeks of starting steroids. Side effects include irritability, stomach irritation, weight gain, difficulty sleeping, mood changes and acne.
When should I use IVIG in ITP?
IVIg is generally recommended for ITP patients under critical bleeding condition, as an emergency rescue procedure, and even for those not responding to GCs or cannot tolerate glucocorticoids (6,23,33).
How long does IVIG last for ITP?
IVIG is given intravenously for a period of several hours. There are two different dosage options: . 4 grams (g) / kilograms (kg) per day for five days, or infusions of 1 g/kg per day for one to two days. The higher-dose, shorter-term administration leads to a more rapid rise in platelet count, but higher toxicities.
How many people with ITP have treatment failure?
Approximately 80% of adult patients with immune thrombocytopenia (ITP) have treatment failure with corticosteroids or become dependent on them and require second-line therapy.
What are therapeutic options?
These therapeutic medical options have variable efficacy, safety profiles, mechanisms of action, and modes of administration. This enables and mandates an individualized approach to treatment, where patient involvement, preferences and values have become central to the process of choosing the appropriate therapy.
How to treat ITP?
Medications to treat ITP may include: 1 Steroids. Your doctor will likely start you on an oral corticosteroid, such as prednisone. Once your platelet count is back to a safe level, you can gradually discontinue taking the drug under the direction of your doctor. Long-term use of these medications isn't recommended because they can increase your risk of infections, high blood sugar and osteoporosis. 2 Immune globulin. If corticosteroids don't help, your doctor may give you an injection of immune globulin. This drug may also be used if you have critical bleeding or need to quickly increase your blood count before surgery. The effect usually wears off in a couple of weeks. 3 Drugs that boost platelet production. Medications such as romiplostim (Nplate) and eltrombopag (Promacta) help your bone marrow produce more platelets. These types of drugs can increase your risk of blood clots. 4 Other drugs. Rituximab (Rituxan, Truxima) helps increase your platelet count by reducing the immune system response that's damaging your platelets. But this drug also can reduce the effectiveness of vaccinations, which may be needed if you later choose surgery to remove your spleen.
What is the best treatment for ITP?
Medications to treat ITP may include: Steroids. Your doctor will likely start you on an oral corticosteroid, such as prednisone. Once your platelet count is back to a safe level, you can gradually discontinue taking the drug under the direction of your doctor.
What are some medications that can help with platelet function?
Examples include aspirin, ibuprofen (Advil, Motrin IB, others) and ginkgo biloba. Medications to treat ITP may include: Steroids.
What is the diagnosis of immune thrombocytopenia?
To diagnose immune thrombocytopenia, your doctor will try to exclude other possible causes of bleeding and a low platelet count, such as an underlying illness or medications you or your child may be taking.
How long does it take for a platelet to wear off?
The effect usually wears off in a couple of weeks. Drugs that boost platelet production. Medications such as romiplostim (Nplate) and eltrombopag (Promacta) help your bone marrow produce more platelets. These types of drugs can increase your risk of blood clots. Other drugs.
What to do if you have thrombocytopenia?
If you have immune thrombocytopenia, try to: Avoid contact sports. Depending on your risk of bleeding, head impacts during sports like boxing, martial arts and football could cause bleeding in your brain. Talk to your doctor about what activities are safe for you. Watch for signs of infection.
What to do if you have a spleen removed?
Watch for signs of infection. If you've had your spleen removed, be alert for any signs of infection, including fever, and seek prompt treatment. Infections can be more serious in people without spleens. Use caution with over-the-counter medications.
What is ITP therapy?
ITP treatment may be conceptually divided into rescue therapy and maintenance therapy . The objective of rescue therapy is a swift rise in platelet count in a patient with active hemorrhage, a high risk for bleeding, or need for a critical procedure. In selecting rescue therapy, a premium is placed on rapidity of response with relatively less regard for durability of response, patient convenience, or safety and tolerability with long-term use. Maintenance therapy, in contrast, is given with the goal of achieving a sustained platelet response while minimizing short- and long-term treatment-related toxicity. Goals and standard treatment options for rescue and maintenance therapy are summarized in Table 1.
What is ITP in a 10 year old?
A 10-year-old male was diagnosed with primary immune thrombocytopenia (ITP) 5 months ago when he presented with epistaxis, petechiae, bruising, and a platelet count of 5 × 10 9 /L. He responded transiently to intravenous immunoglobulin G (IgG), but epistaxis recurred 2 weeks later. He subsequently received a short course of oral corticosteroids to which he had a temporary response in platelet count and cessation of epistaxis. Since discontinuing corticosteroids, he has had only occasional bruising and petechiae. His platelet count is currently 13 × 10 9 /L. He states that ITP is not interfering with activities.
What is the recommended platelet count after splenectomy?
In accordance with American Society of Hematology guidelines, 3 we recommend observation without treatment in most asymptomatic adults after splenectomy with a platelet count of 20 to 30 × 10 9 /L or greater. In a prospective cohort study, patients who maintained a platelet count above this threshold after splenectomy had no bleeding-related deaths. In contrast, patients with a platelet count below 30 × 10 9 /L had a bleeding-related mortality of 36.7%. 11 In a separate study of 47 patients who were unable to maintain a platelet count of 100 × 10 9 /L after splenectomy, there were 3 deaths over a median follow-up of 7.5 years. All 3 had a baseline platelet count below 20 × 10 9 /L. 12 A platelet count threshold above 30 × 10 9 /L may be preferred in some patients because of their occupation, participation in sports, need for antithrombotic therapy, or control of other symptoms that track with platelet count (eg, fatigue, HRQoL). Conversely, some patients have little or no bleeding at platelet counts well below 30 × 10 9 /L and are more concerned about adverse effects of treatment than bleeding. These individuals may elect to defer therapy at lower platelet counts. This is particularly relevant to children, who have low bleeding risk even in the setting of severe persistent or chronic thrombocytopenia. 13 Observation may be appropriate in asymptomatic or minimally symptomatic children, as in Case 1 above, irrespective of the platelet count. We prioritize bleeding symptoms and HRQoL over platelet count in decision-making in this population ( Figure 1 ).
Which TRAs stimulate platelet production?
Thrombopoietin receptor agonists (TRAs) bind to the thrombopoietin receptor and stimulate megakaryocyte maturation and platelet production. Two TRAs, romiplostim and eltrombopag, have been approved by the US Food and Drug Administration. We do not have experience with recombinant human thrombopoietin, which is available in jurisdictions outside the United States.
Is there a gold standard for ITP?
There is no gold standard diagnostic test for ITP. The most compelling evidence for the presence of ITP is a platelet response to standard therapy ( Table 1 ). Nonresponsiveness to treatment should therefore prompt reassessment of the diagnosis and a concerted effort to exclude nonautoimmune causes of thrombocytopenia and secondary ITP. The reader is referred elsewhere for a discussion of diagnostic evaluation. 3, 9, 10 It is likewise imperative that a manual platelet count be performed because automated cell counters may misclassify large or agglutinated platelets, leading to an underestimation of the platelet count and potentially inappropriate treatment decisions.
What is a TTP?
Thrombotic thrombocytopenic purpura (TTP) is the common name for adults with microangiopathic hemolytic anemia, thrombocytopenia, with or without neurologic or renal abnormalities, and without another etiology; children without renal failure are also described as TTP. The diagnosis of TTP is an indication for plasma exchange treatment, ...
What are the histologic abnormalities that are similar to TTP and HUS?
Histologic abnormalities that are similar to TTP and HUS, most often seen in renal biopsies, can also occur in multiple other disorders and cause microangiopathic hemolytic anemia and thrombocytopenia that can mimic the clinical presentation of TTP and HUS. 5.
What is the TTP registry?
The Registry is a population-based cohort of consecutive patients since January 1, 1989 with TTP or HUS identified at the time of a request for plasma exchange treatment. 14, 17 All patients in 58 of Oklahoma's 77 counties are included in the Registry because the Oklahoma Blood Institute is the sole provider of plasma exchange for all hospitals in these counties and because plasma exchange is the standard treatment in this region for all adults diagnosed with TTP or HUS, children who are diagnosed with TTP, and some children diagnosed with HUS. Most children with typical HUS are not included in the Registry because they are not treated with plasma exchange. The Registry enrolled 398 patients with their first episode of clinically diagnosed TTP or HUS through December 31, 2009. ADAMTS13 activity has been measured by Drs Johanna Kremer Hovinga and Bernhard Lämmle (Berne, Switzerland) in serum collected immediately before the first plasma exchange since November 13, 1995 14 ; through December 31, 2009, ADAMTS13 activity was measured in 283 (93%) of 304 patients. ADAMTS13 activity is the same when measured in either serum or plasma. 14 Testing for inhibitors of ADAMTS13 activity was performed on all samples with activities less than 20%. 14 Because we send samples to Berne about twice per year, results are typically available in several months. Severe deficiency of ADAMTS13 activity is defined as less than 10% of normal. 14 The Registry is approved by the Institutional Review Boards of the University of Oklahoma Health Sciences Center and all participating hospitals.
Can sepsis cause thrombocytopenia?
Multiple etiologies of sepsis (bacteria, fungi, rickettsiae, and viruses) can cause thrombocytopenia and microangiopathic hemolytic anemia without signs of DIC. 37 Sepsis suggested by high fever with chills and pulmonary infiltrates, which rarely if ever occur in TTP. Systemic malignancy.
Can plasma exchange be used for aHUS?
However, plasma exchange has been used uncommonly and empirically for children with typical HUS when severe neurologic abnormalities occur. For children with aHUS in whom an abnormality of complement regulation is suspected, current opinion suggests that plasma exchange should be used. 8 , 9.
Do you need a platelet count after remission?
Although routine blood counts are not necessary after several months, a platelet count is absolutely necessary when symptoms of any illness occur, to immediately diagnose a possible recurrence of TTP.
Is pregnancy associated with TTP?
Because pregnancy is a recognized risk factor for triggering acute episodes of TTP, 28 pregnancy is often assumed to be associated with a high risk for a relapse. This assumption seemed to be supported by case reports of subsequent pregnancies associated with recurrent TTP, 58 but case reports may preferentially report dramatic events rather than uncomplicated pregnancies. In the Oklahoma Registry, 27 women have had 51 subsequent pregnancies, including 6 women with severe ADAMTS13 deficiency who have had 12 subsequent pregnancies 58 (data updated through June 30, 2010). Six women (22%) have been diagnosed with TTP during one subsequent pregnancy; these 6 women have also had 9 additional subsequent pregnancies without TTP recurrence. Five of these 6 women were initially diagnosed with TTP before 1995 (therefore, ADAMTS13 was not measured); in 3 the probable cause for thrombocytopenia was preeclampsia. One woman with severe ADAMTS13 deficiency who has had 2 pregnancies after her episode of TTP had a relapse 30 days after her second pregnancy. Therefore, I do not discourage women who have recovered from TTP and who wish to become pregnant, although I discuss the potential risk for a recurrent episode of TTP. I do advise careful management by a specialist in high-risk obstetrics, with platelet counts at each prenatal visit.
What is ITP in a patient?
Patients classified as refractory have a diagnosis that is not really ITP or have disease that is difficult to manage. ITP is a diagnosis of exclusion ; no specific tests exist to confirm the diagnosis. Response to treatment is the only affirmative confirmation of diagnosis. However, refractory patients do not respond to front-line or other treatments; thus, no confirmation of diagnosis exists. The first section of this review carefully evaluates the diagnostic considerations in patients with refractory ITP. The second section describes combination treatment for refractory cases of ITP. The reported combinations are divided into the era before thrombopoietin (TPO) and rituximab and the current era. Current therapy appears to have increased effectiveness. However, the definition of refractory, if it includes insufficient response to TPO agents, describes a group with more severe and difficult-to-treat disease. The biology of refractory ITP is largely unexplored and includes oligoclonality, lymphocyte pumps, and other possibilities. Newer treatments, especially rapamycin, fostamatinib, FcRn, and BTK inhibitors, may be useful components of future therapy given their mechanisms of action; however, TPO agents, notwithstanding failure as monotherapy, appear to be critical components. In summary, refractory ITP is a complicated entity in which a precise specific diagnosis is as important as the development of effective combination treatments.
What is ITP in a diagnosis?
ITP is a diagnosis of exclusion because no specific test defines its presence. 17,18 At diagnosis, recommended laboratory testing is a complete blood cell count (CBC) with differential and review of the smear plus/minus immunoglobulin levels, as well as hepatitis C and HIV testing. The general practice of performing only a limited number of tests creates a higher likelihood of an incorrect diagnosis. In a large series of cases seen by experienced hematologists, the 2 leading misdiagnoses were secondary ITP and myelodysplastic syndrome (MDS). 19,20 Other misdiagnoses included inherited thrombocytopenia, drug-induced thrombocytopenia, and presentation of bone marrow failure with primarily thrombocytopenia ( Tables 1 - 3 ). 21-24 Figure 1 presents an estimate of primary ITP vs other diagnoses in patients thought to have “refractory ITP.” Response to treatments, especially IVIG, is the only criterion allowing diagnosis of ITP with a high degree of certainty (however, the degree of response required to have a high degree of certainty remains ill defined). In contrast, there is no reliable way to confidently diagnose patients with refractory ITP, because (by definition) the patient does not respond to standard ITP treatment. Figure 2 provides a flowchart of the identification and then the diagnosis of refractory ITP.
What is an ITP?
Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder with thrombocytopenia resulting from increased platelet destruction and inhibition of platelet production. 1-4 Most children with ITP have good outcomes with a substantial rate of spontaneous improvement, and those who require intervention or progress to chronic disease usually respond well to treatment. Adults with ITP do not improve as often as children, but they have a higher rate of improvement than generally recognized, perhaps as much as 40% over 1 year and 60% over 3 years. 5 Most patients can usually be managed with conventional treatment. 1,6 However, small groups of patients exist who are very difficult to manage and do not respond to any treatment (ie, have refractory disease).
Can ITP be refractory?
If a case truly appears to be refractory ITP, the authors’ experiences suggest that, in 50% of cases, it may still be another diagnosis, depending upon the experience of the hematologist and the extent of the work-up. However, if it appears to be ITP and multiple single agents have failed to stably increase the platelet count, combination treatments are the next step. These have been explored in ITP but have not been well reviewed.
Is there a confirmation of diagnosis for refractory ITP?
However, refractory patients do not respond to front-line or other treatments; thus, no confirmation of diagnosis exists. The first section of this review carefully evaluates the diagnostic considerations in patients with refractory ITP.
Does rituximab respond to R-CVP?
In addition, patients with no response to rituximab did not respond to R-CVP. In 2007, in the prerituximab era and pre-TPO era, patients who failed to respond to steroids and/or IVIG received induction therapy, followed by combination maintenance therapy.
What is the role of B cells in SLE?
The central role for B cells in the pathogenesis of SLE provides the rationale for the use of the anti-CD20 monoclonal antibody rituximab in its treatment. CD20 is expressed during the intermediate stages of B-cell development but is lost during the terminal stages, being absent on plasma cells. Rituximab is a chimaeric monoclonal antibody against human CD20, and rapidly depletes peripheral blood CD20-positive B cells via complement-mediated and antibody-dependent cellular cytotoxicity [ 105 ]. Although initially used in the treatment of relapsed low-grade B-cell follicular non-Hodgkin’s lymphoma, rituximab was subsequently used successfully in the treatment of chronic ITP [ 106 ].
What are the complications of SLE?
Haematological complications are frequently seen in SLE. Anaemia, leucopenias and thrombocytopenia may result from bone marrow failure or excessive peripheral cell destruction , both of which may be immune mediated. Drugs and infection are other common causes. In this review, we will focus on the diagnosis and management ...
How often should I take CYC for lupus?
CYC therapy (prescribed as 0.75–1.0 g/m 2 body surface area or 10–15 mg/kg), given intravenously every month for at least 4 months appears useful in the treatment of severe lupus-associated thrombocytopenia refractory to standard therapies [ 76 ]. Lower doses given more frequently, for example every 2 weeks, are being used increasingly in some institutions, in order to improve tolerability without loss of efficacy. Balancing the risks and benefits of such therapy can be difficult, particularly in the presence of severe neutropenia. However, successful use of high-dose i.v. CYC in the treatment of aplastic anaemia complicating SLE has been reported [ 77, 78 ]. BM analysis before therapy is particularly helpful in this setting, with increased erythrocyte or leucocyte precursors in the marrow suggesting a response to therapy being more likely. Furthermore, concurrent use of recombinant human G-CSF (rhG-CSF) and antibiotics reduced the risk of this form of approach [ 48 ].
What is eltrombopag used for?
Eltrombopag is a thrombopoeitin receptor agonist in late-stage development for the treatment of thrombocytopenia. Phase II trials have recently been published examining its role in ITP [ 122] and in thrombocytopenia associated with hepatitis C infection [ 123 ]. In the ITP trial, eltrombopag was administered orally at a dose of 30, 50 or 75 mg daily in 118 patients with platelet counts <30 × 10 9 /l for 6 weeks. The primary endpoint was a platelet count >50 × 10 9 /l at Day 43. This was achieved in 28, 70 and 81% in the three eltrombopag dosage groups, respectively, compared with 11% in the placebo group [ 122 ]. The median platelet counts at Day 43 were 26 × 10 9 /l, 126 × 10 9 /l, 183 × 10 9 /l and 16 × 10 9 /l, respectively. In the two higher dosage groups, bleeding episodes were also reduced. Adverse event rates and severity were similar in all four groups. However, the durability of the response remains unclear, and over the 6-week post-treatment follow-up period platelet counts in the eltrombopag groups gradually return to baseline. Notwithstanding, the drug holds promise for SLE-associated thrombocytopenia and no known interactions occur between eltrobompag and the immunosuppressive agents used in SLE.
What is a Blys cytokine?
BLys, a TNF-related cytokine , can be targeted in a number of ways, such as through decoy receptors and with anti-BLyS monoclonal antibodies. LymphoStat-B (belimumab) is a fully human monoclonal antibody that binds to BLyS with high affinity and neutralizes human BLyS bioactivity in vitro and in vivo [ 119 ].
Does SLE cause neutropenia?
The increase in serum TNF-related apoptosis-inducing ligand in SLE may also contribute to neutropenia through excessive neutrophil apoptosis [ 20 ]. Although autoimmunity is the most likely explanation for neutropenia in SLE, other pathologies, such as myelofibrosis, may also on occasion have a role [ 21 ].
Is IVIG effective for lupus?
IVIG can be very effective in some patients with lupus-associated thrombocytopenia. Its use in this context and for other manifestations of SLE has recently been reviewed [ 66 ]. IVIG is well established as a treatment option in ITP, and a long-lasting response in lupus-associated thrombocytopenia has been reported [ 67 ]. Thrombocytopenia and certain other manifestations of SLE, such as neuropsychiatric disease, appear to respond particularly well to IVIG [ 68 ]. Its mechanism of action in SLE is likely to be multimodal. These include down-regulation of autoantibody production, neutralization of pathogenic autoantibodies by anti-idiotypic antibodies, inhibition of complement-mediated damage, modulation of cytokine production, induction of apoptosis in lymphocytes and monocytes, and modulation of both B- and T-lymphocyte function [ 69 ]. Further, in ITP, IVIG may both block activatory and up-regulate inhibitory Fcγ receptors [ 70 ]. Danazol is another option for the specific treatment of thrombocytopenia in SLE [ 71–73 ]. This is generally safe and well tolerated, and like IVIG may be used in pregnancy in this context [ 74 ]. The therapeutic dose of IVIG in severe thrombocytopenia complicating SLE is 2 g/kg, usually given in five consecutive daily doses of 400 mg/kg. Maintenance of remission using repeated doses of IVIG in severe thrombocytopenia complicating SLE has also been reported [ 75 ].
Diagnosis
Treatment
- People with mild immune thrombocytopenia may need nothing more than regular monitoring and platelet checks. Children usually improve without treatment. Most adults with ITPwill eventually need treatment, as the condition often becomes severe or long term (chronic). Treatment may include a number of approaches, such as medications to boost your platelet count or surgery to …
Clinical Trials
- Explore Mayo Clinic studiestesting new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition.
Lifestyle and Home Remedies
- If you have immune thrombocytopenia, try to: 1. Avoid contact sports.Depending on your risk of bleeding, head impacts during sports like boxing, martial arts and football could cause bleeding in your brain. Talk to your doctor about what activities are safe for you. 2. Watch for signs of infection.If you've had your spleen removed, be alert for any...
Preparing For Your Appointment
- Because a low platelet count may not cause symptoms, the problem is often discovered when you have a blood test for another reason. To diagnose immune thrombocytopenia, your doctor is likely to order further blood tests that require drawing a small amount of blood from a vein in an arm. He or she may also refer you to a specialist in blood diseases (hematologist).