What are the International Consensus Report recommendations for the workup of immune thrombocytopenia in children?

Updated: Jan 11, 2020
  • Author: Craig M Kessler, MD, MACP; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
  • Print
Answer

Recommendations for initial investigation of suspected childhood ITP are as follows [40] :

  • Perform a complete history, physical examination, full blood count, and expert analysis of the peripheral blood smear at initial diagnosis.
  • A direct anti-globulin test (DAT) is recommended to exclude coexistent autoimmune hemolytic anemia, especially prior to therapy.
  • Baseline Ig levels, to exclude coexisting immunodeficiency, is recommended prior to therapy.
  • When a child's CBC shows isolated thrombocytopenia, the peripheral blood smear shows no abnormal features beyond thrombocytopenia, and signs of bleeding are present on physical examination, a bone marrow aspiration/biopsy is not required, even prior to steroid therapy.
  • Children with newly diagnosed ITP, especially with atypical features, should be referred to, or their case discussed with, a hematologist experienced in assessment and treatment of children with ITP.
  • Bone marrow aspiration, biopsy, and cytogenetics should be performed if abnormal or potentially malignant cells are visualized on smear and carefully considered if there are other abnormalities of the hemoglobin and/or white cell count (with the exception of microcytic anemia) or if there is hepatosplenomegaly and/or adenopathy. In addition, failure to acutely respond to ITP therapy merits a bone marrow examination.
  • Additional investigations are based on clinical assessment and may include tests insuch as molecular genetics, autoantibody screening, liver-spleen imaging, and other laboratory testing

Recommendations for subsequent investigation of children with persistent or chronic ITP are as follows:

  • Perform a repeat history, physical examination, full blood count, and expert analysis of the peripheral blood smear to reassess the diagnosis.
  • Perform bone marrow aspiration, biopsy, and cytogenetics if no spontaneous platelet increase and no response to treatment has occurred after 3 to 6 months, or earlier if there is no response to treatment within the expected timeframe. Consider next-generation sequencing or targeted sequencing, if available.
  • A bone marrow biopsy is not indicated prior to further therapy (eg, with TPO), but should be included in reevaluation of the diagnosis in the setting of increasingly difficult-to-treat persistent or chronic ITP.

Additional evaluation could include testing for the following:

  • Lupus and other markers of autoimmune diseases that might require specific treatment (eg, test for APLAs, ANAs, anti-cardiolipin antibody, lupus anticoagulant, and serum Igs)
  • Chronic infections (hepatitis, cytomegalovirus, HIV, and/or H pylori in at-risk populations or when there is no other explanation)
  • Complex immunodeficiency diseases
  • Genetic screening for inherited thrombocytopenia and bone marrow failure syndromes

Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!