Immune Thrombocytopenic Purpura (ITP) Guidelines

Updated: Dec 02, 2016
  • Author: Craig M Kessler, MD, MACP; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
  • Print
Guidelines

Guidelines Summary

The American Society of Hematology (ASH) published an updated evidence-based practice guideline for immune thrombocytopenia (ITP) in 2011. [8] The next update of the guideline is expected in 2017. The guideline comprises recommendations (grade 1B) and suggestions (grade 2C). Recommendations and suggestions are provided separately for pediatric and adult patients. In 2013, ASH issued a clinical practice guide on the treatment of thrombocytopenia in pregnancy, based in part on the 2011 guideline. [18]

Next:

Pediatric ITP

Diagnosis

ASH recommendations are that bone marrow examination is not necessary in children and adolescents with the typical features of ITP, or in children in whom intravenous immunoglobulin (IVIg) therapy fails. ASH suggestions are that bone marrow examination is not necessary in similar patients before initiation of treatment with corticosteroids or before splenectomy, and that testing for antinuclear antibodies is not necessary in the evaluation of children and adolescents with suspected ITP. [8]

Initial treatment

ASH has moved away from recommending treatment on the basis of the platelet count. ASH recommends that children with no bleeding or mild bleeding (ie, skin manifestations only, such as bruising and petechiae) be managed with observation alone regardless of platelet count.

Initial treatment

ASH recommendations include the following:

  • First-line treatment for pediatric patients requiring treatment can be a single dose of IVIg (0.8 to 1 g/kg) or a short course of corticosteroids
  • IVIg can be used if a more rapid increase in the platelet count is desired
  • Anti-D therapy is not advised in children whose hemoglobin concentration is decreased because of bleeding, or with evidence of autoimmune hemolysis (grade 1C)

ASH suggests that a single dose of anti-D can be used as first-line treatment in Rh-positive, nonsplenectomized children requiring treatment (grade 2B).

Medical treatment of resistant ITP

For second-line pharmacologic therapy, ASH suggests that rituximab or high-dose dexamethasone may be considered for children or adolescents with ITP who have significant ongoing bleeding despite treatment with IVIg, anti-D, or conventional doses of corticosteroids. Rituximab or high-dose dexamethasone may also be considered as an alternative to splenectomy in children and adolescents with chronic ITP or in patients who do not respond favorably to splenectomy.

Splenectomy

ASH recommends splenectomy for children and adolescents with chronic or persistent ITP who have significant or persistent bleeding and who do not respond to or cannot tolerate other therapies (eg, corticosteroids, IVIg, anti-D), and/or who need improved quality of life.

Given the relatively high rate of spontaneous remission in pediatric ITP, ASH suggests delaying splenectomy or other interventions with potentially serious complications for at least 12 months, unless the patient has severe and unresponsive disease or quality of life concerns that mandate more definitive therapy.

Additional recommendations

ASH also recommends the following:

  • Routine testing for Helicobacter pylori in children with chronic ITP is not indicated
  • Children with a history of ITP who are unimmunized should receive their scheduled first measles-mumps-rubella (MMR) vaccine
Previous
Next:

Adult ITP

Diagnosis

ASH recommends testing adult patients with ITP for hepatitis C virus and HIV. ASH suggests further investigations if the blood count or peripheral blood smear reveals abnormalities other than thrombocytopenia and perhaps findings of iron deficiency. ASH suggests that a bone marrow examination is not necessary irrespective of age in patients presenting with typical ITP. [8]

Initial treatment

ASH suggests that newly diagnosed patients receive treatment if their platelet count is <30 × 109/L. Suggestions for first-line treatment include the following:

  • Longer courses of corticosteroids are preferred over shorter courses of corticosteroids or IVIg
  • IVIg may be used with corticosteroids when a more rapid increase in platelet count is required
  • Either IVIg or anti-D (in appropriate patients) may be used if corticosteroids are contraindicated
  • If used, IVIg should be administered in a single dose of 1 g/kg; this dose may be repeated if necessary

Further therapy

ASH recommendations are as follows:

  • Splenectomy for patients who have failed corticosteroid therapy
  • Thrombopoietin receptor agonists for patients at risk of bleeding who relapse after splenectomy or who have a contraindication to splenectomy and who have failed at least one other therapy

When one line of therapy (eg, corticosteroids, IVIg) has failed and the patient is at risk of bleeding, ASH suggests that the following treatments may be considered:

  • Thrombopoietin receptor agonists, in patients who have not undergone splenectomy
  • Rituximab, after failure of one line of medical therapy or splenectomy

Splenectomy

ASH recommends the following:

  • For medically suitable patients, laparoscopic and open offer similar efficacy
  • Further treatment is not indicated in asymptomatic patients who have platelet counts >30 × 10 9/L after splenectomy
Previous
Next:

Thrombocytopenia in pregnancy

Diagnosis

ASH recommends the following tests for thrombocytopenia in pregnant patients [18] :

  • Complete blood count
  • Reticulocyte count
  • Peripheral blood smear
  • Liver function tests
  • Viral screening (HIV, HCV, HBV)

Tests to consider if clinically indicated include the following:

  • Antiphospholipid antibodies
  • Antinuclear antibody (ANA)
  • Thyroid function tests
  • H pylori testing
  • Disseminated intravascular coagulation testing—prothrombin time (PT), partial thromboplastin time (PTT), fibrinogen, fibrin split products
  • Von Willebrand disease type IIB testing*
  • Direct antiglobulin (Coombs) test
  • Quantitative immunoglobulin levels

The following studies are not recommended:

  • Antiplatelet antibody testing
  • Bone marrow biopsy
  • Thrombopoeitin (TPO) levels

Treatment

Treatment considerations include the following:

  •  Women with no bleeding manifestations and platelet counts ≥30 x 10 9/L do not require any treatment until 36 weeks’ gestation (sooner if delivery is imminent)
  •  If platelet counts are <30 x 10 9/L or clinically relevant bleeding is present, first-line therapy is oral corticosteroids or intravenous immunoglobulin (IVIg)
  • The recommended starting dose of IVIg is 1 g/kg
  • Prednisone and prednisolone are preferred to dexamethasone, which crosses the placenta more readily.
  • Recommended starting doses of prednisone by different experts vary from 0.25 to 0.5 to1 mg/kg daily;no evidence exists that a higher starting dose is better
  • Medications are adjusted to maintain a safe platelet count

Expected responses to first-line therapy are as follows:

  • Oral corticosteroids—initial response 2-14 days, peak response 4-28 days
  • IVIg—initial response 1-3 days, peak response 2-7 days

Second-line therapy for refractory ITP is with combined corticosteroids and IVIg or, in the second trimester, splenectomy.  For third-line therapy, anti-D immunoglobulin and azathioprine are relatively contraindicated. Agents that are not recommended, but whose use in pregnancy has been described, include the following:

  • Cyclosporine
  • Dapsone
  • Thrombopoietin receptor agonists
  • Campath-1H
  • Rituximab

Contraindicated agents include the following:

  • Mycophenolate mofetil
  • Cyclophosphamide
  • Vinca alkaloids
  • Danazol

Management at the time of delivery

ASH recommendations are as follows:

  • Because of the possible need for cesarean delivery, the recommended target platelet count prior to labor and delivery is ≥50 x 10 9/L
  • A woman whose platelet count is <80 x 10 9/L but who has not required therapy during pregnancy can be started on oral prednisone (or prednisolone) 10 days prior to anticipated delivery at a dose of 10-20 mg daily and titrated as necessary
  • The mode of delivery should be determined by obstetric indications
  • Although the minimum platelet count for the placement of regional anesthesia is unknown and local practices may differ, many anesthesiologists will place a regional anesthetic if the platelet count is ≥80 x 10 9/L
  • While platelet transfusion alone is generally not effective in ITP, its use in conjunction with IVIg can be considered if an adequate platelet count has not been achieved and delivery is emergent
  • Percutaneous umbilical blood sampling (PUBS) or fetal scalp blood sampling is not recommended, as it is not helpful in predicting neonatal thrombocytopenia and is potentially harmful
  • In the newborn, the nadir platelet count reaches its nadir 2-5 days after delivery and rises spontaneously by day 7
  • Postpartum thromboprophylaxis should be considered, as women with ITP are at increased risk of venous thromboembolism
Previous