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

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.)

In view of uncontrolled observations and anecdotal reports suggesting that the activated PCC, Autoplex, was much more effective than standard (non-activated) PCC in controlling bleeding in hemophiliacs with inhibitors, a controlled double-blind study was designed to compare the effectiveness of Autoplex and Proplex. Acute hemarthrosis was chosen for study as this common but non-life-threatening lesion lends itself well to controlled study. A single dose of “unknown” product (Autoplex 75 FECU/kg; Autoplex 50 FECU/kg; or Proplex 75 FIX U/kg) was given, and effectiveness was judged at 6 hr. By all criteria of efficacy, there were no significant differences between the products. It is noteworthy that a single dose of PCC was judged effective in 50% of episodes, a figure that is consistent with other published clinical trials. In this model, no additional benefit was derived from using the activated PCC, Autoplex, in either dosage.

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