Immune thrombocytopenia, previously known as idiopathic thrombocytopenic purpura (ITP), is an autoimmune disease that is related to anti-platelet immunoglobulin (IgG) production. The production of IgG autoantibodies is critically dependent on cellular immune mechanisms particularly relating to T cells. The production of these autoantibodies by B cells depends on a number of cellular mechanisms that form a network of modulation, with T cells playing a pivotal role in pathophysiology. T-cell-mediated cytotoxicity is an alternative mechanism for platelet destruction in ITP [1-3]. Other causes are included genetic factors (immune genes-FcR, immune syndromes, platelet antigens) and susceptibility to initial event (infection, inflamination) . Immune thrombocytopenia is characterised by increased mucocutaneous haemorrhage risk with low platelet counts .
Purpura are not seen in many patients, and ITP was shortened to immune thrombocytopenia. The diagnosis of immune thrombocytopenia is made via the exclusion of other factors that cause thrombocytopenia. The frequency of ITP in adults per year is 1.6-6.6 per 100,000 (6).
The incidence of ITP in adults is approximately equal for the sexes except in the mid-adult years (30-60 years), when the disease is more prevalent in women [7-9]. The initial hypothesized cause of ITP was increased platelet destruction at a rate that exceeded production by compensating bone marrow. New knowledge questions this model and provides evidence that platelet production is also decreased in many ITP patients . There is no “gold standard” test that reliably establishes the diagnosis. However, a positive response to an ITP-specific therapy, e.g., intravenous immunoglobulin (IVIg) and/or steroids, is supportive of the diagnosis.
The stages of immune thrombocytopenia were re-defined:
The primary target of ITP treatment is the prevention of major haemorrhage by attaining a safe thrombocyte count, but not the return of thrombocyte counts to normal. A level of 50 x 109/L may be sufficient in ITP patients with generally good functioning platelets. Adults with platelet counts less than 30 × 109/L are generally treated. Multiple large cohort studies reported that patients with platelet counts above that level were safely observed without treatment [10,11].
The incidence of haemorrhagic diathesis increases with age , and the effect of this condition on quality of life [3,14], mortality and morbidity should be considered [15,16]. Studies of co-morbidities and risk factors in large series of ITP patients with long follow-ups are rare [17,18]. Studies of surgical complications and mortality were only conducted for splenectomy procedures (19). The global features of adverse postoperative outcomes for ITP patients undergoing all types of surgical procedures have not been studied in a population-based cohort [20,21].