成人免疫介导的血小板减少症:治疗进展

Md. Rafiquzzaman Khan
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摘要

免疫性血小板减少症(ITP)是由免疫介导的血小板破坏和/或血小板产生受损引起的获得性血小板计数低。原发性ITP是特发性的,继发性ITP与其他疾病有关。发病率为每10万人中有1至6人。itp可能没有症状或伴有出血。出血表现为轻微(皮肤/粘膜)、严重(如颅内、眼内、腹膜后、肌肉内出血等)和严重(血红蛋白下降2 g/dL或需要输血2单位红细胞)。ITP的诊断依据是孤立性血小板减少症而无贫血或白细胞减少症。没有可靠的实验室检查来确认诊断。治疗ITP的目的是提供一个安全的血小板计数,以防止出血,而不是使血小板计数正常化。ITP的治疗方案有一线治疗、二线治疗和综合治疗。一线治疗是严重出血和血小板计数30 × 10^9/L,有出血风险,其他止血缺陷或需要手术的患者。自发性缓解发生在高达10%的成人ITP。孟加拉国J医学2023;第34卷,第2(1)增编:189-190
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Immune-Mediated Thrombocytopenia in adults: Treatment Updates
Immune Thrombocytopenia (ITP) is an acquired low platelet count resulting from immune-mediated platelet destruction and/ or impaired platelet production. Primary ITP is idiopathic and secondary ITP is associated with another conditions. The incidence is 1 to 6 per 100,000 adults. ITPmay present without symptom or present with bleeding. Bleeding manifestations are minor (skin/mucous membrane), critical (e.g. intracranial, intraocular, retroperitoneal, intramuscular bleeding etc) and severe (fall in hemoglobin of 2 g/dL or requires transfusion of e”2 units of red cells).ITP is diagnosed on the basis of isolated thrombocytopenia without anemia or leukopenia. There are no reliable laboratory tests to confirm the diagnosis. The aim of treatment of ITP is to provide a safe platelet count to prevent bleeding, rather than to normalize the platelet count. Treatment options of ITP are the Firstline therapies, second-line therapies and therapies. First-line therapy are for patients with severe bleeding and platelet count <30x10^9/L, which includes platelet transfusion, glucocorticoids (e.g. methylprednisolone, 1 g IV, daily for 3 doses; or dexamethasone, 40 mg orally or IV, daily for four days), IVIG and IV globulin. Second-line therapy is indicated for patients with thrombocytopenia associated with significant bleeding or for severe, persistent or recurrent thrombocytopenia (e.g., platelet count <20x10^9/L) following glucocorticoid-based treatments. Secondline therapies include splenectomy, rituximab, thrombopoietin receptor agonist or immunosuppressive therapy. Other therapies include danazol, vincristine, procarbazine, etoposide etc, or combination therapy. Indications of treatment are (1).Severe bleeding and platelet count <30x10^9/L. (2). Newly diagnosed ITP and any clinically important bleeding (3) Newly diagnosed ITP and platelet count <20x10^9/L, even in absence of bleeding (4) Patients with platelet counts >30x10^9/L having risk of bleeding, other hemostatic defects or require surgery.Spontaneous remission occurs in up to 10% of adults with ITP. Bangladesh J Medicine 2023; Vol. 34, No. 2(1) Supplement: 189-190
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