начните с того, как лечат детей во всем мире: преднизолон - первая линия, как и ввиг, дексаметазон пульс - вторая линия;
надеюсь, специалисты по переводу мед. статей в Москве еще остались?
Therapy
About two out of three pediatric patients with ITP show a spontaneous improvement in platelet count in 6 months without necessity of medical treatment, and those remissions are usually sustained. Most of patients with newly diagnosed ITP do not show signs of bleeding, and can be managed with a “watch and see” strategy (83–86).
There is no absolute consensus about the platelet threshold necessary to start treatment in ITP: 1996 guidelines of the American Society of Hematology recommended to treat patients with a platelet count lower than 10,000/μl and minor purpura or those one with a count lower 20,000/μl and significant bleeding (87). An update published in 2011 suggested that children without bleeding or with mild bleeding should be managed only with observations, regardless of platelet count (88). Despite these recommendations, most patients with low risk of bleeding are currently treated (89).
First-line Treatment
Prednisone–Prednisolone
All guidelines support the use of corticosteroids in the first-line treatment of ITP. Oral prednisone is often effective in inducing response in pediatric patients when administered at doses of 1–2 mg/kg for 7–14 days and maintains efficacy also at higher doses (4 mg/kg/day) for 3 or 4 days, raising platelet count over 50,000/μl in the first 72 h in 72–88% of patients (78, 90, 91).
However, due to the adverse effects of a prolonged treatment with corticosteroids in children, those drugs must be used only for short periods, to maintain a hemostatic platelet count (78).
Intravenous Immunoglobulins (IVIg)
Immunoglobulins have been used for ITP since 1981 (92, 93), for the effect of modulation on immune system. The treatment induces a raise in platelet count in 80% of pediatric patients, obtaining an effect in the first 48 h more frequent than corticosteroids (94). IVIg are usually administered in a single dose of 0.8–1 g/kg, with the chance of using a second dose in case of incomplete response, even if also lower doses (0.6 g/kg) are reported to be effective (95). Adverse effects include headache and fever and are more common when used doses are greater than 1 g/kg for consecutive days (91).
Intravenous Anti-D Immunoglobulin
Rh-positive children could receive short infusions of anti-D immunoglobulin, with a recommended dose of 50–75 µg/kg (78). This therapeutic strategy has a response rate greater than 50% and acts more rapidly than IVIG (76, 77, 96, 97).
However, in patients with comorbidity, the treatment has been associated with severe hemolysis, acute renal failure, and disseminated intravascular coagulation, and therefore anti-D immunoglobulin administration should require a careful selection of patients and post-therapy monitoring, as concluded by Despotovic et al. (98).
Second-line Therapies
High-Dose Corticosteroids
High-dose methylprednisolone has been used as an alternative to IVIg, showing comparable response rates (99, 100).
Dexamethasone (28–40 mg/m2/day) has been used in pediatric patients with chronic refractory ITP, obtaining response rates greater than 80%, with and a mean duration of the response of 26 months (101): moreover, psychiatric adverse effects, such as insomnia and aggressive behavior, are extremely frequent (102), and this makes dexamethasone only a second-line therapeutic alternative.
Rituximab
This anti-CD20 antibody, used in other autoimmune diseases and B-cell lymphoma, has been used in chronic refractory ITP often showing response rates greater than 60% (103–106), even though in a study by Bennett et al. only 31% of patients responded (107). However, follow-up studies have shown that sustained response is uncommon (108, 109), and safety profile is unclear.
Danazol
This attenuated androgen is successfully used in second-line treatment of adult patients with ITP, particularly in elderly patients (110). There are only a few studies about its use in pediatric patients, showing a good effectiveness without significant adverse reactions. Unfortunately, danazol can accelerate bone growth, and this limits its applicability in prepuberal patients (111, 112).
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The Centenary of Immune Thrombocytopenia—Part 2: Revising Diagnostic and Therapeutic Approach.