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血液和肿瘤病

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免疫性血小板减少性紫癜(ITP)
原作者: Haney Mallemat, 肖锋译 发布日期:2013-08-14

Title: Immune Thrombocytopenia Purpura (ITP)
题目:免疫性血小板减少性紫癜(ITP)
Author 作者: Haney Mallemat

Keep Immune Thrombocytopenic Purpura (ITP) in your differential for patients with thrombocytopenia and evidence of bleeding. Although ITP has classically been described in children, it can occur in adults; especially between 3rd- 4th decade.
对有血小板减少和出血证据的患者,要将免疫性血小板减少性紫癜(ITP)做为你的一个鉴别诊断。尽管ITP被传统地描述为儿童疾病,它可以发生在成人,尤其是第三,四十年龄段。
Thrombocytopenia leads to the extravasation of blood from capillaries, leading to skin bruising, mucus membrane petechial bleeding, and intracranial hemorrhage.
血小板减少导致血液从毛细血管渗出,出现皮肤青紫,粘膜瘀点状出血,和颅内出血。
ITP occurs from production of auto-antibodies which bind to circulating platelets. This leads to irreversible uptake by macrophages in the spleen. Causes of antibody production include:
ITP的发生由于产生结合循环中血小板的自身抗体,这导致脾巨噬细胞(对血小板的)不可逆转的摄取。抗体产生的原因包括:
Medication exposure
药物
Infection (usually viral), including HIV and hepatitis
感染(通常是病毒感染),包括艾滋病毒和肝炎
Immune disorders (e.g., lupus)
免疫疾病(如红斑狼疮)
Pregnancy
怀孕
Idiopathic
特发性

Suspect ITP in patients with isolated thrombocytopenia on a CBC without other blood-line abnormalities. Abnormality in other blood-line warrants consideration of another diagnosis (e.g., leukemia).
对于一个CBC显示血小板减少但没有其他血象异常的患者要怀疑ITP。如有其他血象异常值得考虑另外的诊断 (如白血病)。

ITP cannot be cured; treatments include:
ITP不能治愈,治疗方法包括:
Steroid to suppress antibody production (first-line therapy)
类固醇以抑制抗体的产生(一线治疗)
Intravenous immunoglobulin (IVIG)
静脉注射免疫球蛋白(IVIG)
IV Rho immunoglobulin (for Rh+ patients only)
静脉用Rho免疫球蛋白(只限于RH(+)患者 )
Rituximab +/- dexamethasone
利妥昔单抗+/ - 地塞米松
Splenectomy (rare cases of massive hemorrhage refractory to pharmacologic treatment)
脾切除术(罕见的对药物治疗无效的大出血病例)