Treatment FAQ

what is the current treatment for ttp

by Garfield Brown Published 3 years ago Updated 2 years ago
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The current standard of treatment for the acute episode of TTP involves plasma exchange (PEX) and steroids.2, 3 Patients that are refractory to this treatment are offered rituximab most commonly,4, 5 although other therapies including cyclosporine and cyclophosphamide have been used successfully in the past.Dec 18, 2017

Is there a natural remedy for TTP and Hus?

Is there a natural remedy for TTP and HUS. A 39-year-old member asked: Is there a natural remedy for ttp and hus? Dr. Haleh Agdassi answered. Physical Medicine and Rehabilitation 26 years experience. Not that I am aware: I do not believe there is a natural remedy for this condition.

What does TTP mean medically?

Your doctor will also check your blood for:

  • antibodies, which are proteins that interfere with the enzyme ADAMTS13
  • a lack of ADAMTS13 enzyme activity, which causes TTP
  • the enzyme lactate dehydrogenase, which is released from tissue injured by blood clots that are caused by TTP
  • high creatinine levels, because TTP can cause kidney problems (which affects the kidneys’ ability to filter creatinine)

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Is TTP curable?

There are several methods for treating TTP. These include medications as well as lifestyle changes and alternative treatments. A doctor may recommend that you experiment with a combination of approaches to help manage the condition. The sections below will discuss some of these approaches.

How do you get TTP?

You can get acquired TTP in a variety of ways. You can develop it if you have HIV , for example. You can also develop it after certain medical procedures, such as a blood and marrow stem cell ...

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How is TTP 2020 treated?

PEX and corticosteroids are the mainstays of treatment of acquired TTP. Individuals with thrombocytopenia and MAHA, and without secondary causes of TMA, meet the working diagnosis of acquired TTP.

What is the best treatment for TTP?

Plasma treatments 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.

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.

How long is TTP treatment?

Most people who have plasma therapy for TTP will need several days up to a few weeks of healing time. Those who have a splenectomy usually need about four to six weeks to recover.

What is the prognosis for TTP?

The overall response rate to plasma exchange is 75-90%. The early mortality rate is 10-20%. Long-term survival depends largely on the presence or absence of serious underlying comorbidities such as cancer, HIV infection, or solid organ transplantation.

How long can you live with TTP?

The most striking evidence for the impact of morbidities following recovery from TTP is decreased survival. Among the 77 patients who survived their initial episode of TTP (1995-2017), 16 (21%) have subsequently died, all before their expected age of death (median difference, 22 years; range 4-55 years).

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 FFP given in TTP?

Fresh frozen plasma is used for management and prevention of bleeding, as a coagulation factors replacement, and to treat thrombotic thrombocytopenic purpura (TTP).

How does rituximab work for TTP?

PEX is essential in TTP treatment as plasma contains the missing enzyme ADAMTS 13. Rituximab (licensed and internationally used monoclonal antibody) selectively acts on white blood cells known as B-lymphocytes or B cells that produce the antibody to ADAMTS 13.

Is TTP curable?

Annually, there are four new cases of TTP per 100,000 people. It is most common in adults from 20-50 years old, and it affects women slightly more often than it affects men. There is no known cure.

Can you survive TTP?

WHAT HAPPENS FOLLOWING RECOVERY FROM AN EPISODE OF TTP? Before we talk about recovery, we must say that some patients with TTP still die. Most of our patients who died never had a chance for effective treatment; patients who have begun plasma exchange treatment have almost always survived.

What triggers TTP?

A lack of activity in the ADAMTS13 enzyme (a type of protein in the blood) causes thrombotic thrombocytopenic purpura (TTP). The ADAMTS13 gene controls the enzyme, which is involved in blood clotting. Not having enough enzyme activity causes overactive blood clotting.

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.

Overview

Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder. With TTP, thrombi ( blood clots) develop in small vessels throughout your body. These thrombi can restrict the flow of blood to your body’s vital organs, including the heart, kidneys and brain. As a result, serious medical problems can arise.

Symptoms and Causes

People with TTP may experience a number of warning signs. Common thrombotic thrombocytopenic purpura symptoms include:

Diagnosis and Tests

Your healthcare provider will perform a physical examination and ask about your symptoms and health history. If your provider suspects TTP, they will order diagnostic tests.

Management and Treatment

In most cases, plasma therapy is used to treat TTP. Other options include medication and surgery.

Prevention

Both types of TTP develop suddenly with no clear cause. As a result, it’s not possible to prevent it. However, if you’ve had TTP, you can talk to your healthcare provider about ways to reduce your risk of flare-ups.

Living With

You should schedule a visit with your healthcare provider anytime you notice TTP symptoms, such as fatigue, shortness of breath or purplish spots on your skin. If you’ve already been diagnosed with TTP, watch out for relapse symptoms. In most cases, your provider will restart plasma therapy to get your symptoms under control again.

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and ischemic end organ injury due to microvascular platelet-rich thrombi.

1. Introduction

In 1924, Dr. Eli Moschcowitz described a previously healthy 16-year-old girl who became acutely ill with fever, weakness, focal neurological symptoms, and severe thrombocytopenia. Ultimately, she became comatose and died after one week.

2. Epidemiology

iTTP typically presents in adulthood, accounting for 90% of cases [ 29 ]. The annual incidence is 1.5–6 cases per million per year in adults [ 29, 30, 31, 32 ]. Discrepancies in annual incidence rate are likely due to demographic factors in the country of origin.

3. Pathophysiology

ADAMTS13 is a critically important enzyme, synthesized in hepatic stellate cells [ 43, 44 ], whose only known function is to regulate VWF multimers [ 9, 10 ]. In physiologic conditions, ADAMTS13 is in a latent, closed conformation and VWF, secreted by platelets and endothelial cells, is in a globular state ( Figure 1 a) [ 45, 46 ].

4. Diagnosis

Previously, TTP was defined by a clinical “pentad” consisting of fever, microangiopathic hemolytic anemia, thrombocytopenia, neurological deficits, and renal insufficiency [ 4 ]. However, the pentad was reported at a time before the effectiveness of plasma-based therapy in treating TTP was firmly established.

5. Acute Management

TTP is a clinical emergency and in patients with suspected TTP treatment should be initiated promptly as delays in therapy may result in significant morbidity and mortality. Often therapy decisions are required prior to the availability of confirmatory ADAMTS13 testing.

6. Special Populations

TTP in the pregnant patient presents many difficulties and challenges. These patients should be managed by a multidisciplinary team typically including hematologists, high-risk obstetricians, and, occasionally, neonatologists.

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