Ghosh Jayaditya, Dharamdasani Satyender, Parthan Girish, Shah Ravi, Dutta Pinaki
Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh, IND.
Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh, IND.
Cureus. 2024 Jun 13;16(6):e62328. doi: 10.7759/cureus.62328. eCollection 2024 Jun.
Sheehan's syndrome (SS) is a condition characterized by panhypopituitarism that generally occurs after an episode of postpartum bleeding. There are certain hypotheses regarding the development of SS in the postpartum period. Coagulation factor abnormalities have been reported to be associated with SS. Associated hypothyroidism and hypocortisolism have been found to cause coagulation abnormalities. After the correction of the hypothyroidism and hypocortisolism, there is a gradual correction of the coagulation abnormality. In our case, a middle-aged woman presented with recurrent episodes of hospital admission because of generalized weakness and fever. She was found to have a biochemistry profile suggestive of hypopituitarism with preserved gonadal function. Her hemogram was normal, but the coagulogram showed a prolongation of activated partial thromboplastin time with a near-normal prothrombin time. She was evaluated and found to have factor XI deficiency. In the background of excessive vaginal bleeding and hypopituitarism, a diagnosis of SS was made. The presence of factor XI deficiency may have led to excessive bleeding and the development of SS. To the best of our knowledge, there is no reported association of factor XI deficiency with SS in the literature, and this is the first reported case.
希恩综合征(SS)是一种以全垂体功能减退为特征的病症,通常发生在产后出血事件之后。关于产后时期SS的发展有某些假说。据报道,凝血因子异常与SS有关。已发现相关的甲状腺功能减退和皮质醇缺乏会导致凝血异常。在纠正甲状腺功能减退和皮质醇缺乏后,凝血异常会逐渐得到纠正。在我们的病例中,一名中年女性因全身无力和发热反复入院。发现她的生化检查结果提示垂体功能减退但性腺功能保留。她的血常规正常,但凝血检查显示活化部分凝血活酶时间延长而凝血酶原时间接近正常。对她进行评估后发现有因子XI缺乏。在大量阴道出血和垂体功能减退的背景下,诊断为SS。因子XI缺乏的存在可能导致了大量出血和SS的发生。据我们所知,文献中没有报道因子XI缺乏与SS的关联,这是首例报道病例。