Table 2.

Recommendations from 2011 ASH guideline for ITP that are not addressed in the 2019 ASH guideline on ITP

ITP in adults 
 Newly diagnosed ITP in adults 
  Initial diagnosis of ITP 
   4.1.A. We recommend: 
    • Testing patients for HCV and HIV (grade 1B*
   4.1.B. We suggest: 
    • Further investigations if there are abnormalities (other than thrombocytopenia and perhaps findings of iron deficiency) in the blood count or smear (grade 2C) 
    • A bone marrow examination is not necessary irrespective of age for patients presenting with typical ITP (grade 2C) 
  First-line treatment of adult ITP 
   4.3.A. We suggest: 
    • IVIG be used with corticosteroids when a more rapid increase in platelet count is required (grade 2B) 
    • Either IVIG or anti-D (in appropriate patients) be used as a first-line treatment if corticosteroids are contraindicated (grade 2C) 
    • If IVIG is used, the dose should initially be 1 g/kg as a 1-time dose; this dosage may be repeated if necessary (grade 2B) 
  Laparoscopic vs open splenectomy and vaccination prior to splenectomy 
   4.5.A. We recommend: 
    • That for medically suitable patients, both laparoscopic and open splenectomy offer similar efficacy (grade 1C) 
 Treatment of ITP in pregnancy 
  Management of ITP during pregnancy 
   6.1.A. We recommend: 
    • Pregnant patients requiring treatment receive either corticosteroids or IVIG (grade 1C) 
  Treatment of ITP during labor and delivery 
   6.2.A. We suggest: 
    • For pregnant women with ITP, the mode of delivery should be based on obstetric indications (grade 2C) 
 Treatment of specific forms of secondary ITP 
  Management of secondary ITP, HCV-associated 
   7.1.A. We suggest: 
    • For patients with secondary ITP due to HCV infection, antiviral therapy should be considered in the absence of contraindications (grade 2C); however, the platelet count should be closely monitored due to a risk of worsening thrombocytopenia attributable to interferon 
    • ITP is required, the initial treatment should be IVIG (grade 2C) 
  Management of secondary ITP, HIV-associated 
   7.2.A. We recommend: 
    • For patients with secondary ITP due to HIV, treatment of the HIV infection with antiviral therapy should be considered before other treatment options unless the patient has clinically significant bleeding complications (grade 1A) 
    • If treatment of ITP is required, initial treatment should consist of corticosteroids, IVIG, or anti-D (grade 2C) and splenectomy in preference to other agents in symptomatic patients who fail corticosteroids, IVIG, or anti-D (grade 2C) 
  Management of secondary ITP, H pylori–associated 
   7.3.A. We recommend: 
    • That eradication therapy be administered for patients who are found to have H pylori infection (based on urea breath tests, stool antigen tests, or endoscopic biopsies) (grade 1B) 
   7.3.B. We suggest: 
    • Screening for H pylori be considered for patients with ITP in whom eradication therapy would be used if testing is positive (grade 2C) 
ITP in children 
 Newly diagnosed ITP in children 
  Diagnosis of ITP 
   1.1.A. We recommend: 
    • Bone marrow examination is unnecessary in children and adolescents with the typical features of ITP (grade 1B) 
    • Bone marrow examination is not necessary in children who fail IVIG therapy (grade 1B) 
   1.1.B. We suggest: 
    • Bone marrow examination is also not necessary in similar patients prior to initiation of treatment with corticosteroids or before splenectomy (grade 2C) 
    • Testing for antinuclear antibodies is not necessary in the evaluation of children and adolescents with suspected ITP (grade 2C) 
 Children who are treatment nonresponders 
  H pylori testing in children with persistent or chronic ITP 
   2.3.A. We recommend: 
    • Against routine testing for H pylori in children with chronic ITP (grade 1B) 
 Management of MMR-associated ITP 
   3.1.A. We recommend: 
    • Children with a history of ITP who are unimmunized receive their scheduled first MMR vaccine (grade 1B) 
    • In children with either nonvaccine or vaccine-related ITP who have already received their first dose of MMR vaccine, vaccine titers can be checked; if the child displays full immunity (90% to 95% of children), then no further MMR vaccine should be given; if the child does not have adequate immunity, then the child should be reimmunized with MMR vaccine at the recommended age (grade 1B) 
ITP in adults 
 Newly diagnosed ITP in adults 
  Initial diagnosis of ITP 
   4.1.A. We recommend: 
    • Testing patients for HCV and HIV (grade 1B*
   4.1.B. We suggest: 
    • Further investigations if there are abnormalities (other than thrombocytopenia and perhaps findings of iron deficiency) in the blood count or smear (grade 2C) 
    • A bone marrow examination is not necessary irrespective of age for patients presenting with typical ITP (grade 2C) 
  First-line treatment of adult ITP 
   4.3.A. We suggest: 
    • IVIG be used with corticosteroids when a more rapid increase in platelet count is required (grade 2B) 
    • Either IVIG or anti-D (in appropriate patients) be used as a first-line treatment if corticosteroids are contraindicated (grade 2C) 
    • If IVIG is used, the dose should initially be 1 g/kg as a 1-time dose; this dosage may be repeated if necessary (grade 2B) 
  Laparoscopic vs open splenectomy and vaccination prior to splenectomy 
   4.5.A. We recommend: 
    • That for medically suitable patients, both laparoscopic and open splenectomy offer similar efficacy (grade 1C) 
 Treatment of ITP in pregnancy 
  Management of ITP during pregnancy 
   6.1.A. We recommend: 
    • Pregnant patients requiring treatment receive either corticosteroids or IVIG (grade 1C) 
  Treatment of ITP during labor and delivery 
   6.2.A. We suggest: 
    • For pregnant women with ITP, the mode of delivery should be based on obstetric indications (grade 2C) 
 Treatment of specific forms of secondary ITP 
  Management of secondary ITP, HCV-associated 
   7.1.A. We suggest: 
    • For patients with secondary ITP due to HCV infection, antiviral therapy should be considered in the absence of contraindications (grade 2C); however, the platelet count should be closely monitored due to a risk of worsening thrombocytopenia attributable to interferon 
    • ITP is required, the initial treatment should be IVIG (grade 2C) 
  Management of secondary ITP, HIV-associated 
   7.2.A. We recommend: 
    • For patients with secondary ITP due to HIV, treatment of the HIV infection with antiviral therapy should be considered before other treatment options unless the patient has clinically significant bleeding complications (grade 1A) 
    • If treatment of ITP is required, initial treatment should consist of corticosteroids, IVIG, or anti-D (grade 2C) and splenectomy in preference to other agents in symptomatic patients who fail corticosteroids, IVIG, or anti-D (grade 2C) 
  Management of secondary ITP, H pylori–associated 
   7.3.A. We recommend: 
    • That eradication therapy be administered for patients who are found to have H pylori infection (based on urea breath tests, stool antigen tests, or endoscopic biopsies) (grade 1B) 
   7.3.B. We suggest: 
    • Screening for H pylori be considered for patients with ITP in whom eradication therapy would be used if testing is positive (grade 2C) 
ITP in children 
 Newly diagnosed ITP in children 
  Diagnosis of ITP 
   1.1.A. We recommend: 
    • Bone marrow examination is unnecessary in children and adolescents with the typical features of ITP (grade 1B) 
    • Bone marrow examination is not necessary in children who fail IVIG therapy (grade 1B) 
   1.1.B. We suggest: 
    • Bone marrow examination is also not necessary in similar patients prior to initiation of treatment with corticosteroids or before splenectomy (grade 2C) 
    • Testing for antinuclear antibodies is not necessary in the evaluation of children and adolescents with suspected ITP (grade 2C) 
 Children who are treatment nonresponders 
  H pylori testing in children with persistent or chronic ITP 
   2.3.A. We recommend: 
    • Against routine testing for H pylori in children with chronic ITP (grade 1B) 
 Management of MMR-associated ITP 
   3.1.A. We recommend: 
    • Children with a history of ITP who are unimmunized receive their scheduled first MMR vaccine (grade 1B) 
    • In children with either nonvaccine or vaccine-related ITP who have already received their first dose of MMR vaccine, vaccine titers can be checked; if the child displays full immunity (90% to 95% of children), then no further MMR vaccine should be given; if the child does not have adequate immunity, then the child should be reimmunized with MMR vaccine at the recommended age (grade 1B) 

H pylori, Helicobacter pylori; HCV, hepatitis C virus; MMR, measles, mumps, and rubella.

*

Evidence grades: The number value indicates the strength of the recommendation. A value of 1 indicates a high degree of confidence that the desirable outcomes of an intervention exceed the undesirable outcomes effects (or vice versa) in most patient populations. A value of 2 indicates a lower degree of confidence that the desirable outcomes outweigh undesirable outcomes (or vice versa). The letter score indicates the quality of the underlying evidence. “A” indicates that the recommendation is supported by consistent evidence from RCTs or exceptionally strong observational studies. “B” indicates that the recommendation is supported by RCTs with important limitations or strong evidence from observational studies. “C” indicates evidence derived from RCTs with serious flaws, weaker observational studies, or indirect evidence.42 

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