Table 1

Proposed definitions of disease

Primary ITP Primary ITP is an autoimmune disorder characterized by isolated thrombocytopenia (peripheral blood platelet count <100 × 109/L) in the absence of other causes or disorders that may be associated with thrombocytopenia. The diagnosis of primary ITP remains one of exclusion; no robust clinical or laboratory parameters are currently available to establish its diagnosis with accuracy. The main clinical problem of primary ITP is an increased risk of bleeding, although bleeding symptoms may not always be present. 
Secondary ITP All forms of immune-mediated thrombocytopenia except primary ITP* 
Phases of the disease Newly diagnosed ITP: within 3 months from diagnosis 
 Persistent ITP: between 3 to 12 months from diagnosis. Includes patients not reaching spontaneous remission or not maintaining complete response off therapy. 
 Chronic ITP: lasting for more than 12 months 
 Severe ITP: Presence of bleeding symptoms at presentation sufficient to mandate treatment, or occurrence of new bleeding symptoms requiring additional therapeutic intervention with a different platelet-enhancing agent or an increased dose 
Primary ITP Primary ITP is an autoimmune disorder characterized by isolated thrombocytopenia (peripheral blood platelet count <100 × 109/L) in the absence of other causes or disorders that may be associated with thrombocytopenia. The diagnosis of primary ITP remains one of exclusion; no robust clinical or laboratory parameters are currently available to establish its diagnosis with accuracy. The main clinical problem of primary ITP is an increased risk of bleeding, although bleeding symptoms may not always be present. 
Secondary ITP All forms of immune-mediated thrombocytopenia except primary ITP* 
Phases of the disease Newly diagnosed ITP: within 3 months from diagnosis 
 Persistent ITP: between 3 to 12 months from diagnosis. Includes patients not reaching spontaneous remission or not maintaining complete response off therapy. 
 Chronic ITP: lasting for more than 12 months 
 Severe ITP: Presence of bleeding symptoms at presentation sufficient to mandate treatment, or occurrence of new bleeding symptoms requiring additional therapeutic intervention with a different platelet-enhancing agent or an increased dose 
*

The acronym ITP should be followed by the name of the associated disease (for thrombocytopenia after exposure to drugs, the terms “drug-induced” should be used) in parentheses: for example, “secondary ITP (lupus-associated),” “secondary ITP (HIV-associated),” and “secondary ITP (drug-induced).” For manuscript titles, abstracts, and so on, definitions such as lupus-associated ITP or HIV-associated ITP can also be used.

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