Idiopathic thrombocytopenic purpura: a practice guideline developed by explicit methods for the American Society of Hematology [see comments]

Abstract

DIOPATHIC thrombocytopenic purpura (ITP, also I known as primary immune thrombocytopenic purpura) is a hematologic disorder for which appropriate diagnostic and treatment strategies are uncertain. In 1994, the American Society of Hematology (ASH) established a panel to produce explicitly developed practice guidelines for the diagnosis and management of ITP. "Explicitly developed," evidencebased practice guidelines, which are being issued increasingly by medical specialty societies, combine a critical appraisal of scientific evidence with practice recommendations that state clearly to what extent the guidelines are based either on published scientific evidence or opinion (eg, clinical experience).I4 More details about the clinical practice guideline movement are provided elsewhere.'.'This report begins with a brief summary of the panel's recommendations, followed by a more detailed analysis of its methodology, the findings of the comprehensive literature review, and a full presentation of the recommendations. The report concludes with recommendations for future research. As explained later, the recommendations are based on the panel's opinion, derived from a systematic scoring methodology. (Only recommendations receiving scores of 1 .O to 3.0 or 7.0 to 9.0, as defined later in the text, are cited in this summary.) SUMMARY OF RECOMMENDATIONS Children Diagnosis

Outcome and management in neonatal thrombocytopenia due to maternal idiopathic thrombocytopenic purpura

“…We can conclude that despite severe neonatal thrombocytopenia due to maternal ITP during pregnancy was fairly high in our series, severe bleeding manifestations were rare. As platelet nadir is seen within the first 7 days postpartum, bleeding risks are the highest in the first week, and daily platelet count control is necessary until a stable or rising safe platelet count above 50 × 10 9 /l is observed (as advised in guidelines) . In addition should each individual case be evaluated precisely, to determine whether daily platelet count measurement is really necessary or once in 2 days will suffice.…”

Is flow cytometry accurate enough to screen platelet autoantibodies?

“…Although the ASH guidelines stated in 1996 that PLT‐associated IgG assay was unnecessary and inappropriate in both adults and children ITP patients, the British guidelines considered in 2003 that the investigation of PLT autoantibodies may be of value in adult ITP patients in particular clinical settings, that is, combination of marrow failure and ITP or ITP patients refractory to first‐ and second‐line treatment for whom a third‐line treatment is considered 1,13 . Moreover, several authors also state that antibody testing is helpful for positive diagnosis (rather than to make only a diagnostic of exclusion) and may be used in the follow‐up of patients to determine immune remission or a worsening outcome in ITP 2,3,14 .…”

“…ITP is classified as “primary” or “idiopathic” when occurring without any underlying disease or considered as “secondary” when associated with various dysimmune disorders, such as systemic lupus erythematosus or lymphoproliferative diseases. According to the American Society of Hematology (ASH) guidelines, “the diagnosis is based principally on the history, physical examination, complete blood count, and examination of the peripheral smear, which should exclude other causes of thrombocytopenia” and not on the detection of PLT autoantibodies 1 . More recent publications suggest that laboratory tests could be incorporated in current guidelines 2,3 .…”

Platelet transfusion practices in immune thrombocytopenia related hospitalizations

“…Case series have reported short term increases in platelet counts following platelet transfusions with amelioration of massive hemorrhage and the prevention of bleeding during invasive surgery, thus providing a rationale for their use in patients with hemorrhage or who are at high risk for bleeding . Published guidelines recommend that the use of platelet transfusions in management of ITP be limited to patients with life‐threatening hemorrhage or those in whom an invasive surgical procedure is planned …”

“…We observed platelet transfusions in less than 5% of all admissions in children with ITP. This may reflect the benign course of ITP in pediatric patients, with spontaneous remission in nearly four‐fifths of the children and the risk of medical complications including ICH, GI bleed, and mortality being associated with older age at presentation …”