<h2>ITP treatment guidelines for adults1-3</h2>
The ideal treatment for ITP varies between different age groups. Therefore, healthcare providers must consider the patient’s age when preparing a treatment plan. Below is a summary of ASH recommendations on how to treat ITP in adults.
<a href="https://ashpublications.org/bloodadvances/article/3/23/3829/429213/American-Society-of-Hematology-2019-guidelines-for" target="_blank">ASH recommendations</a> mainly cover primary immune thrombocytopenia guidelines, where the primary cause of ITP is unknown. In secondary ITP, the cause of the disease (eg, an infection or medication) is known and first-line treatment often addresses the pre-existing condition.&nbsp;

<h2>First-line ITP treatment1</h2>
Key decisions for healthcare providers treating ITP are criteria for admitting a patient to the hospital and when to start treatment.&nbsp;
Newly diagnosed patients with a platelet count below 20 (x109/L) without life-threatening bleeding should be admitted to the hospital, whilst patients with an established ITP diagnosis or a platelet count exceeding 20 (x109/L) can be treated at home.&nbsp;
Not all patients with ITP require medical interventions. Asymptomatic patients with non-life-threatening bleeding and a platelet count exceeding 30 (x109/L) can be managed with observation. In contrast, first-line treatment should be considered for all patients when their platelet count falls below 30 (x109/L).&nbsp;
<h2>Management of adult ITP patients with non-life-threatening bleeding1:</h2>
<figure class="table" style="width:100%;">
 <table class="ck-table-resized">
 <colgroup>
 <col style="width:17.51%;">
 <col style="width:41.16%;">
 <col style="width:41.33%;">
 </colgroup>
 <tbody>
 <tr>
 <td>&nbsp;</td>
 <td style="text-align:center;" colspan="2">Initial pharmacological intervention vs observation</td>
 </tr>
 <tr>
 <td>Clinical criteria</td>
 <td style="text-align:center;">Platelet count&nbsp; 
 &lt;30 (x109/L) 
 in 
 Newly diagnosed ITP patients 
 who are asymptomatic or have minor 
 mucocutaneous bleeding</td>
 <td style="text-align:center;">Platelet count&nbsp; 
 ≥30 (x109/L) 
 in 
 Newly diagnosed ITP patients 
 who are asymptomatic or have minor 
 mucocutaneous bleeding</td>
 </tr>
 <tr>
 <td>Management recommendation</td>
 <td style="text-align:center;">Start of first-line treatment</td>
 <td style="text-align:center;">Observation</td>
 </tr>
 <tr>
 <td>&nbsp;</td>
 <td style="text-align:center;" colspan="2">Hospital admission vs home management</td>
 </tr>
 <tr>
 <td>&nbsp;</td>
 <td style="text-align:center;">Platelet count&nbsp; 
 &lt;20 (x109/L) 
 in 
 Newly diagnosed ITP patients 
 who are asymptomatic or have minor 
 mucocutaneous bleeding</td>
 <td style="text-align:center;">Platelet count&nbsp; 
 ≥20 (x109/L) 
 in 
 Newly diagnosed ITP patients 
 who are asymptomatic or have minor 
 mucocutaneous bleeding</td>
 </tr>
 <tr>
 <td>&nbsp;</td>
 <td style="text-align:center;">Inpatient treatment</td>
 <td style="text-align:center;">Outpatient treatment</td>
 </tr>
 </tbody>
 </table>
</figure>

<a href="${link:{uuid:{5951058e-a726-4620-a2dc-66df25bdf733},repository:{articles},path:{/Global/indications/itp/treating-itp}}}">First-line treatments for ITP</a> include corticosteroids, intravenous immunoglobulin (IVIg), and anti-D immunoglobulin. Corticosteroids are deemed a suitable first-line treatment for most ITP patients. Importantly, all first-line treatment decisions should consider the patient’s preferences.1,2,4 &nbsp;
The impact of ITP on patients goes beyond platelet burden and can severely impact a person’s quality of life. The treatment therapies or treatment modalities of ITP can also adversely affect patients. For instance, corticosteroids can cause hypertension and negatively influence sleep and mental health.1,2,4
Treating healthcare providers should consider regularly assessing patients living with ITP and their health-related quality of life (HRQoL). Learn more about the <a href="${link:{uuid:{9d762c77-664a-467f-a4f0-78211ab9453e},repository:{articles},path:{/Global/indications/itp/living-with-itp}}}">HRQoL impacts of patients living with ITP</a>.1,2,4&nbsp;

<h2>Advanced therapy use for ITP</h2>
Some patients experience ITP for more than 3 months or do not respond to corticosteroids.1
For these patients, a change in the treatment plan should be considered. <a href="${link:{uuid:{5951058e-a726-4620-a2dc-66df25bdf733},repository:{articles},path:{/Global/indications/itp/treating-itp}}}">Second-line ITP treatment</a>&nbsp;options include rituximab, splenectomy, and thrombopoietin receptor agonists (TPO-RA).1
The guidelines provide comprehensive considerations for selecting the best second-line treatments for ITP. Patients who are opposed to long-term medication may benefit from a splenectomy or treatment with rituximab. However, ITP patients can spontaneously go into remission within the first year,1 in which case a splenectomy should be avoided in this period. On the other hand, TPO-RAs might be a suitable option for patients who place a high value on achieving a durable response or avoiding surgery.1
In patients with additional risk factors, some treatments may elevate the risk of thrombosis and thromboembolism.5,6
<h2>Considerations influencing treatment discussions1:</h2>
<ul>
 <li>Adherence to medication</li>
 <li>Age of the patient</li>
 <li>Availability</li>
 <li>Comorbidities</li>
 <li>Cost</li>
 <li>Duration of the disease</li>
 <li>Frequency of bleeding</li>
 <li>Patient preferences</li>
</ul>
Overall, more treatment options are needed in ITP. Response rates and durability of current treatments can vary widely whilst some also have unfavourable side effect profiles or dosing regimens. Learn more about <a href="${link:{uuid:{8eff7d37-c8d4-477c-a6b5-52e04965a9b6},repository:{articles},path:{/Global/indications/itp/unmet-needs}}}">the future of ITP treatments</a>.7,8&nbsp;

<h2>ITP treatment algorithm</h2>
Making a second-line treatment plan for ITP is complex as patient preferences and different clinical factors must be considered.1,2&nbsp;
This treatment algorithm for adult patients can help provide general guidance on managing persistent or first-line treatment-resistant ITP. 1,2&nbsp;

Algorithm for the selection of second-line therapy in adults with ITP. Selection of second-line therapy in adults with ITP should be individualised based on duration of disease and patient values and preferences. Other factors that may influence treatment decisions include frequency of bleeding sufficient to require hospitalisation or rescue medication, comorbidities, compliance, medical and social support networks, cost, and availability of treatments. Patient education and shared decision-making is encouraged. Numbered recommendations corresponding to each treatment option are provided.1

<h2>Limitations of the ASH ITP treatment guidelines for adults</h2>
The guidelines only cover patients who have been already diagnosed with ITP and exclude guidance on treatments approved after 2017 and patients who are pregnant or haemorrhaging.1
<h2>Do other ITP guidelines agree?</h2>
Guidelines for ITP can differ slightly between countries. However, most guidelines are aligned on treatment approaches, including2,6:
<ul>
 <li>An emphasis on incorporating patient preference into treatment decisions and discussing the potential impact of different treatments on quality of life.</li>
 <li>Acknowledging that preferences of patients with chronic ITP may evolve during the course of the disease and recommending ongoing discussions as a routine part of follow-up.</li>
</ul>

ASH, American Society of Hematology; IVIg, intravenous immunoglobulin; HRQoL, health-related quality of life; ITP, immune thrombocytopenic purpura.

The American Society of Hematology (ASH) guidelines provide evidence-based guidance on the management of adult patients with newly diagnosed and chronic immune thrombocytopenia (ITP). This guidance was delivered by an ITP expert panel consisting of clinical specialists and patients.1

ITP Treatment Guidelines

IMMUNE-THROMBOCYTOPENIC-ITP

<h2>In a study, 75% of ITP patients felt their condition was not well-managed from a HRQoL perspective.3</h2>
Current treatment goals in ITP are to stop active bleeding and reduce the risk of further bleeding. Response rates for ITP treatments vary widely as measured by improved platelet counts, and the mechanisms of action (MOA) of current treatments may not fully address the complex nature of the disease.1
Additional rescue medication is often necessary for patients with ITP.4

—Female living with ITP, UK

<h2>Investigational therapies for ITP</h2>
Ongoing research aims to better address the multiple dimensions of ITP by targeting the underlying pathophysiology of the disease. Several clinical trials are in progress investigating novel drugs for treating ITP.5,6

Bruton’s tyrosine kinase (BTK) inhibitors, which inhibits macrophage phagocytosis leading to a decrease in platelet destruction, prevent the production of autoantibodies, and may inhibit inflammation5,7-9

Neonatal Fc receptor (FcRn) blockers, which increase platelet antibody clearance, leading to a decrease in peripheral platelet destruction and immune response5,10

Recombinant immunoglobulin multimers, which decrease platelet destruction and increase antiplatelet antibody clearance6

Complement inhibitors, which decrease complement-dependent cytotoxicity11

Plasma-cell–depleting therapies, which decrease antiplatelet antibodies6,11

B cell activating factor (BAFF) inhibitors, which reduce B cell survival and consequently decrease antiplatelet antibodies12,13

<h3>Bruton’s tyrosine kinase (BTK) inhibitors5,7-9</h3>
BTK is an enzyme expressed mainly in B cells, myeloid cells, and platelets. BTK is crucial for the signalling pathways that regulate the development and maturation of B cells and the overall immune response.
Several BTK inhibitors targeting these mechanisms have been approved or are currently in development. BTK inhibitor (BTKi) therapy can prevent macrophage phagocytosis in ITP, leading to a decrease in platelet destruction; can prevent production of autoantibodies; and may also inhibit inflammation.
However, patients can develop a resistance to BTK inhibitors when mutations in the targeted cell occur. Newer generations of BTK inhibitors aim to tackle BTK inhibitor resistance and common class AEs with various strategies.&nbsp;
<h3>Neonatal Fc receptor (FcRn) blockers5,10</h3>
FcRn regulates the immune response by presenting immunoglobulin G (IgG) antigens to immune cells and regulating IgG levels in the plasma.
FcRn blockers in ITP may increase platelet antibody clearance, leading to a decrease in peripheral platelet destruction and immune response.&nbsp;
<h3>Recombinant immunoglobulin multimers6</h3>
Recombinant immunoglobulin multimers decrease platelet destruction and increase antiplatelet antibody clearance. Recombinant immunoglobulin multimers bind to Fc receptors on immune cells, preventing the attachment of pathogenic antibodies to these receptors. As a consequence, in conditions like ITP, the immune-mediated destruction of platelets could be reduced and platelet counts increase.

<h3>Complement inhibitors6,11</h3>
Complement inhibitors decrease complement-dependent cytotoxicity. Complement inhibitors block the activation of the complement system, a part of the immune response that marks cells, such as platelets, for destruction. By inhibiting specific complement pathways, these agents may potentially reduce platelet destruction in autoimmune conditions like ITP.&nbsp;
<h3>Plasma-cell–depleting therapies6,11</h3>
Plasma-cell–depleting therapies decrease antiplatelet antibodies. Plasma-cell–depleting therapies target and reduce plasma cells, helping to minimise platelet destruction and improve platelet counts. However, plasma-cell–depleting therapies may fail to completely eliminate long-lived plasma cells that regulate antiplatelet autoantibody production.&nbsp;
<h3>B cell activating factor (BAFF) inhibitors12,13</h3>
BAFF binds to its receptor on B cells, promoting the survival of B cells and initiating the differentiation of the B cells to immunoglobulin-producing plasma cells. Both are crucial factors in ITP pathogenesis.
BAFF inhibitors may reduce B cell survival and consequently decrease antiplatelet antibodies. Thus, in conditions like ITP, BAFF inhibitors could help to preserve platelet counts.
AE, adverse event; Fc, fragment crystallisable; HRQoL, health-related quality of life.

Diagnosing ITP

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New treatment options are needed for patients with immune thrombocytopenia (ITP).1,2

Unmet Needs

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ITP Treatment Guidelines

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