Saxena R, Mishra D K, Kashyap R, Choudhry V P, Mahapatra M, Bhargava M
Department of Haematology, All India Institute of Medical Sciences, New Delhi, India.
Haemophilia. 2000 Mar;6(2):78-83. doi: 10.1046/j.1365-2516.2000.00372.x.
Clinico-haematological features in 10 patients with acquired Haemophilia are presented. Three patients had FVIII inhibitors following pregnancy while in six the cause for development of inhibitors could not be determined. One patient had acquired von Willebrand's disease. Lupus anticoagulant coexisted with Factor VIII inhibitors in three patients. All patients presented with sudden onset of bleeding without any past or family history of a bleeding disorder. Factor VIII inhibitor levels ranged from 8 to 512 Bethesda units in the nine patients. Immunosuppressive therapy was given to 8 patients, consisting of CVP regimen or corticosteroids with endoxan or cyclosporin. Seven patients had clinical and laboratory responses and one patient did not respond. One patient had severe postpartum bleeding with acute shock which was controlled with FEIBA. Diagnosis and management of idiopathic acquired Haemophilia, thus, continues to be a major challenge, and among acquired Haemophilia, postpartum Haemophilia has good prognosis.
本文介绍了10例获得性血友病患者的临床血液学特征。3例患者在妊娠后出现FVIII抑制物，6例患者抑制物产生的原因无法确定。1例患者患有获得性血管性血友病。3例患者狼疮抗凝物与因子VIII抑制物共存。所有患者均突然出现出血症状，既往及家族均无出血性疾病史。9例患者的因子VIII抑制物水平在8至512贝塞斯达单位之间。8例患者接受了免疫抑制治疗，包括CVP方案或使用环磷酰胺或环孢素的皮质类固醇。7例患者有临床和实验室反应，1例患者无反应。1例患者产后严重出血并伴有急性休克，通过活化凝血酶原复合物浓缩剂（FEIBA）得以控制。因此，特发性获得性血友病的诊断和治疗仍然是一项重大挑战，在获得性血友病中，产后血友病预后良好。
