What is the treatment for thrombotic thrombocytopenic purpura?
Treatment of Thrombotic Thrombocytopenic Purpura. In most cases, TTP occurs suddenly and lasts for days or weeks, but it can go on for months. Relapses (flareups) can occur in up to 60 percent of people who have acquired TTP. Flareups also occur in most people who have inherited TTP. Plasma treatments are the most common way to treat TTP.
What are the treatment options for relapsing thrombocytopenia (TTP)?
Other interventions. Splenectomy: The role of splenectomy in the current management of TTP is limited. Historically, it has been performed for relapsing disease; however, there is little good evidence regarding its efficacy and safety. Rituximab should be considered the mainstay of treatment for relapsing disease.
Which medications are used in the treatment of toxic pulmonary thrombosis (TPT)?
PEX, methylprednisolone, and rituximab are the mainstays of the acute management of acquired TTP; however, other important aspects of supportive care include:
What recommendations did the panel make for treatment of idiopathic thrombocytopenic purpura (ittp)?
The panel agreed on 11 recommendations based on evidence ranging from very low to moderate certainty. For first acute episode and relapses of iTTP, the panel made a strong recommendation for adding corticosteroids to therapeutic plasma exchange (TPE) and a conditional recommendation for adding rituximab and caplacizumab.
What medication is used for TTP?
Medicines used to treat TTP include glucocorticoids, vincristine, rituximab, and cyclosporine A. Sometimes surgery to remove the spleen (an organ in the abdomen) is needed. This is because cells in the spleen make the antibodies that block ADAMTS13 enzyme activity.
What is the first line treatment for acute TTP?
The first-line therapy for acute TTP is based on daily therapeutic plasma exchange supplying deficient ADAMTS13, with or without steroids. Additional immune modulators targeting ADAMTS13 autoantibodies are mainly based on steroids and the humanized anti-CD20 monoclonal antibody rituximab.
What is the most effective treatment for bleeding secondary to thrombotic thrombocytopenic purpura?
Plasma exchange with fresh frozen plasma is the therapy of choice for TTP. Octaplas is a pooled plasma (human) that has been treated with a solvent detergent process. This blood product provides a viable alternative to single-donor fresh-frozen plasma, with a reduced risk of certain viral transmissions.
Does prednisone help with purpura?
Prednisone is a Prescription Therapy to treat Immune Thrombocytopenic Purpura (ITP)
How is thrombotic thrombocytopenia treated?
Therapeutic plasma exchange (plasmapheresis) is used to treat acquired TTP. In this procedure, the liquid part of your blood (plasma) is replaced with donor plasma, using a machine that collects the cells in the blood. It removes antibodies (proteins) in your blood that damage your ADAMTS13 enzyme.
Is rituximab Approved for TTP?
Treatment with rituximab normalizes ADAMTS13 and induces durable remissions in >90% of patients with refractory or relapsing TTP. Rituximab is increasingly used in the upfront setting with evidence of improved rates of remission and disease-free interval.
Why is platelet contraindicated in TTP?
Platelet transfusions are typically not recommended for patients with TTP because they have been hypothesized to provoke fatal thrombotic events.
Is bleeding time increased in TTP?
Because TTP causes increased clotting, more blood platelets are used up. This can cause internal bleeding, bleeding underneath the skin and other bleeding problems.
Are steroids used to treat thrombocytopenia?
Corticosteroids have been used for more than 30 years as a first-line treatment for adult immune thrombocytopenia (ITP). The most common regimen is oral prednisone 1 mg/kg/d, slowly tapering to the lowest possible dose (based on platelet count) during a period of weeks.
Can steroids treat ITP?
Two corticosteroids that may be prescribed for ITP are high-dose dexamethasone and oral prednisone (Rayos). According to 2019 guidelines from the American Society of Hematology (ASH), adults newly diagnosed with ITP should be treated with prednisone for no more than 6 weeks.
Does dexamethasone help platelets?
Dexamethasone (Decadron) is designed to increase the platelet count by slowing the destruction of platelets. Oral courses of highdose dexamethasone temporarily suppresses the body's immune system so that it stops destroying platelets. Dexamethasone may help your bone marrow cells make more platelets.
What is the role of ADAMTS13 in TTP?
The key role of ADAMTS13 deficiency in TTP pathophysiology naturally suggests that administration of recombinant ADAMTS13 (rADAMTS13) would be a promising strategy. In vitro, rADAMTS13 addition to the plasma of 2 patients with TTP and congenital ADAMTS13 deficiency led to a dose-dependent normalization of vWF-cleaving activity, resulting in the degradation of vWF multimers. 30 rADAMTS13 also prevented TTP development in a ADAMTS13 knockout mouse model. 31 These promising experimental data strongly suggest that rADAMTS13 administration could become a valuable therapeutic option in TTP. In line with this statement, a phase I clinical trial is underway to evaluate the safety and pharmacokinetics of rADAMTS13 in children and young adults with congenital TTP (BAX930; https://clinicaltrials.gov/ct2/show/NCT02216084?term=ADAMTS13+baxter&rank=1 ). However, in patients with acquired TTP, anti-ADAMTS13 autoantibodies could bind to the infused rADAMTS13, inhibit its activity, and accelerate its clearance. This could compromise its therapeutic efficacy. The administration of high doses of rADAMTS13 may overcome this problem. The synthesis of functionally active rADAMTS13 variants that are resistant to the inhibitory effect of autoantibodies may represent an alternative solution that needs to be validated in clinical trials. 32
What is the survival rate of adamets13?
Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of 80%-85% . However, relapses occur in ∼40% of patients and refractory disease with fatal outcomes still occurs. In this context, the introduction of rituximab has probably been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, or even as frontline therapy, with high response rates. In more severe patients, salvage strategies may include twice-daily TPE, pulses of cyclophosphamide, vincristine, as well as splenectomy in more desperate cases. In this life-threatening disease, relapse prevention represents a major goal. Persistent severe acquired ADAMTS13 deficiency in patients who are otherwise in remission is associated with a high risk of relapse and preemptive treatment with rituximab may be considered in this context. In the coming years, the TTP therapeutic landscape should be enriched by original strategies stemming from clinical experience and new agents that are currently being evaluated in large, ideally international, clinical trials. Promising agents under evaluation include N -acetylcysteine, bortezomib, recombinant ADAMTS13, and inhibitors of the glycoprotein-Ib/IX-von Willebrand factor axis.
What is TPE in TTP?
The standard treatment of acquired TTP consists mainly of daily therapeutic plasma exchange (TPE) that allows ADAMTS13 repletion and, to a lesser extent, removal of anti-ADAMTS13 antibodies and possibly pro-aggregatory substances.
How long does it take for rituximab to work?
Importantly, rituximab might not be effective for the treatment of unresponsive TTP during the first 2 weeks, with a reported delay in the onset of its effect that may reach 27 days. 7
What is NAC used for?
N -acetylcysteine (NAC), which is known to reduce mucin multimers, has been proposed as an adjunctive treatment for TTP because vWF multimers have some common structural features with mucin multimers. Indeed, in vitro NAC reduces soluble vWF multimers and rapidly degrades ultra-large vWF multimers. In addition, administration of NAC to ADAMTS13–deficient mice reduces plasma vWF multimers and leads to rapid resolution of thrombi. 25 Recently, the use of NAC as adjunctive treatment in a patient with refractory TTP was apparently efficient. 26 However, some failures were also reported. 27, 28 Considering the limited toxicity of NAC, its use as adjunctive treatment in TTP seems interesting and needs to be further evaluated.
What antiplatelet agents are used in TTP?
The use of antiplatelet agents in TTP is based on the rationale that the main pathological feature of the disease is the presence of platelet thrombi in the microcirculation. Aspirin and dipyridamole are the 2 main compounds considered to be potentially effective for TTP treatment. However, in TTP, the main mechanism of platelet clumping involves the glycoprotein Ib-IX/von Willebrand factor (vWF) axis. This challenges the relevance of aspirin and dipyridamole in TTP treatment because their mechanism of action involves other pathways. This discrepancy may account for the variability in the tolerance and results reported by the clinical trials that evaluated the efficacy of these antiplatelet agents in TTP (for a review, see Coppo and Veyradier 1 ).
What is the name of the drug that is used to treat multiple myeloma?
Bortezomib. Bortezomib (Velcade, Millenium Pharmaceuticals), an inhibitor of the 26S unit of the proteasome, is used in the treatment of multiple myeloma and antibody-mediated rejection of solid organs.
What is the most common treatment for TTP?
Relapses (flareups) can occur in up to 60 percent of people who have acquired TTP. Flareups also occur in most people who have inherited TTP. Plasma treatments are the most common way to treat TTP. Other treatments include medicines and surgery. Treatments are done in a hospital.
What is plasma therapy?
Plasma Therapy. Plasma is the liquid part of your blood. It carries blood cells, hormones, enzymes, and nutrients to your body. TTP is treated with plasma therapy. This includes: Fresh frozen plasma for people who have inherited TTP. Plasma exchange for people who have acquired TTP.
How long does thrombocytopenic purpura last?
In most cases, TTP occurs suddenly and lasts for days or weeks, but it can go on for months.
How long does it take for plasma to be frozen?
The time required to complete the procedure varies, but it often takes about 2 hours. Treatments of fresh frozen plasma or plasma exchange usually continue until your blood tests results and signs and symptoms improve.
How does plasma exchange work?
During plasma exchange, an IV needle or tube is placed in a vein in your arm to remove blood. The blood goes through a cell separator, which removes plasma from the blood. The nonplasma part of the blood is saved, and donated plasma is added to it.
Drugs used to treat Thrombotic Thrombocytopenic Purpura
The following list of medications are in some way related to, or used in the treatment of this condition.
Further information
Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.
What is the therapy for thrombocytopenic purpura?
The therapy of choice is plasma exchange with fresh frozen plasma and immunosuppression. In January 2019 the FDA approved caplacizumab (Cablivi) for adults with acquired thrombotic thrombocytopenic purpura (aTTP), in combination with plasma exchange and immunosuppressive therapy. It has been shown to reduce time to platelet count response ...
Does Rituximab reduce relapses?
A study by Froissart and colleagues, in 22 adult patients who had responded poorly to plasma exchange, found that treatment with rituximab resulted in shorter overall treatment duration and reduced 1-year relapses , compared with historical controls. [ 22]
Is dipyridamole used for TTP?
Although used in the past, aspirin and dipyridamole are no longer used in treating TTP. The anti-CD20 monoclonal antibody rituximab has been reported to have activity in TTP that is refractory to plasma exchange.
Is vincristine a chemotherapeutic agent?
The chemotherapeutic agent vincristine has been used as an adjunct to plasma exchange in patients with refractory disease, but its routine use has not been validated.