Guidelines Summary
In 2019, the American Society of Hematology (ASH) published an update to their 2011 evidence-based practice guideline for immune thrombocytopenia (ITP). The 2019 guideline comprises strong recommendations and conditional recommendations/suggestions. Recommendations and suggestions are provided separately for pediatric and adult patients. [18] Some of the 2011 recommendations remained unchanged and were not included in the 2019 review and update. [16]
Pediatric ITP
The 2011 ASH guidelines recommend 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. [16]
ASH has moved away from recommending treatment on the basis of the platelet count. The 2019 ASH guidelines recommend 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. [18]
ASH suggestions include the following for children with non–life-threatening mucosal bleeding and/or diminished health-related quality of life (HRQoL) [18] :
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A short course of corticosteroids over anti-D immunoglobulin or IVIG for first-line treatment
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Thrombopoietin receptor agonists (TPO-RAs) over rituximab or splenectomy for second-line treatment
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Rituximab over splenectomy for second-line treatment
Adult ITP
The 2011 ASH guidelines recommend 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. [16]
The 2019 ASH guidelines recommend against treatment of patients with a platelet count ≥30 × 109/L. For newly diagnosed patients with a platelet count < 30 × 109/L, guidelines suggest treatment with corticosterioids. For patients with a platelet count < 20 × 109/L, the guidelines suggest hospital admission for treatment. For first-line treatment, the guidelines further recommend a short course (≤6 weeks) of steroids over a prolonged course (> 6 weeks including treatment and taper) of steroids. [18]
Additional first-line treatment suggestions include the following [18] :
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Either prednisone (0.5-2.0 mg/kg per day) or dexamethasone (40 mg/day for 4 days) as the corticosteroid
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Corticosteroids alone rather than rituximab and corticosteroids
The following 2011 ASH guidelines treatment suggestions remain unchanged [16] :
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IVIG may be used with corticosteroids when a more rapid increase in platelet count is required
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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; the dose may be repeated if necessary
Second-line treatment suggestions include the following [18] :
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Either splenectomy or a TPO-RA.
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In adults who are going to be treated with aTPO-RA, either eltrombopag or romiplostim
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Rituximab over splenectomy
The 2011 ASH recommendation that for medically suitable patients laparoscopic and open splenectomy offer similar efficacy also remains unchanged. [16]
