Fukuda T, Akimoto J, Chin M, Itoh H, Miwa T, Hasue M
Department of Neurosurgery, Tokyo Medical College.
No Shinkei Geka. 1990 Jun;18(6):511-20.
Therapies and prognoses covering fifteen cases of intracranial hematoma (ICrH) accompanying various types of bleeding tendency (BTD) were studied along with a secondary analysis of the pertinent references. Fifteen cases were divided into two groups, Group A comprising 11 cases of ICrH accompanying primary BTD, and Group B comprising four cases of ICrH accompanying secondary BTD caused by various underlying diseases. Group A included four cases of hemophilia A (Hp-A), two cases of factor XIII deficiency (FXIII-d), three cases of thrombocytopenia (Th-p) and two cases of vitamin K deficiency (VK-d). The four cases of Hp-A responded favorably, with good prognoses, to a supplementary therapy alone. This result was endorsed by the development of therapy as documented in the references. The combined five cases of FXIII-d and Th-p tended without exception, to show good prognoses in the wake of a combination therapy of supplementary treatment and surgical procedure. As regards FXIII-d, there was an inter-reference difference in supplementary doses. Many references shared the view that splenectomy was essential to the treatment of Th-p in general, and idiopathic thrombocytopenic purpura in particular. The current study also suggested that gammaglobulin in large doses would serve as an effective therapy. The two cases of VK-d suffered from a serious degree of lingering neurologic manifestations, although their lives were saved. Even though there is an established therapy for it, VK-d was found to be a problem with poor functional prognosis showing the importance of the preventive approach. Group B was classified into the acute type and the subacute type depending on the rate of pathologic development. As underlying diseases DIC and myelofibrosis due to acute myeloblastic leukemia, and Th-p due to aplastic anemia were noted in two cases in each group. Of these, two cases of the subacute type were able to be saved, while two cases of the acute type followed poor prognostic courses resulting, eventually, in death. The following were found to be responsible fatal factors: 1) causes of BTD which involved both mechanisms of coagulation and hemostasis, 2) non-removal of the underlying disease, in which case supplementary therapy tended to be futile, and 3) the underlying disease per se as a danger to the life of the patient. In conclusion, therapeutic rationale and prognosis in ICrH accompanying primary type of BTD will benefit from the implementation of an adequate augmentative therapy as in the ordinary type of ICrH.(ABSTRACT TRUNCATED AT 400 WORDS)
对15例伴有各种出血倾向(BTD)的颅内血肿(ICrH)患者的治疗方法和预后进行了研究,并对相关参考文献进行了二次分析。15例患者分为两组,A组包括11例伴有原发性BTD的ICrH患者,B组包括4例由各种基础疾病引起的继发性BTD的ICrH患者。A组包括4例甲型血友病(Hp-A)、2例因子 XIII 缺乏症(FXIII-d)、3例血小板减少症(Th-p)和2例维生素K缺乏症(VK-d)。4例Hp-A患者仅接受补充治疗后反应良好,预后良好。参考文献中记录的治疗进展证实了这一结果。FXIII-d和Th-p合并的5例患者在补充治疗和手术联合治疗后无一例外预后良好。关于FXIII-d,补充剂量在参考文献之间存在差异。许多参考文献一致认为,脾切除术对一般Th-p,尤其是特发性血小板减少性紫癜的治疗至关重要。本研究还表明,大剂量丙种球蛋白将是一种有效的治疗方法。2例VK-d患者虽保住了性命,但遗留严重的神经功能表现。尽管已有针对VK-d的既定疗法,但发现其功能预后较差,这表明预防措施很重要。B组根据病理发展速度分为急性型和亚急性型。每组各有2例患者的基础疾病为急性髓细胞白血病引起的弥散性血管内凝血(DIC)和骨髓纤维化,以及再生障碍性贫血引起的Th-p。其中,2例亚急性型患者得以挽救,而2例急性型患者预后不良,最终死亡。发现以下是致命因素:1)涉及凝血和止血机制的BTD病因;2)基础疾病未消除,在这种情况下补充治疗往往无效;3)基础疾病本身对患者生命构成危险。总之,原发性BTD伴发的ICrH的治疗原理和预后将受益于像普通类型ICrH那样实施适当的强化治疗。(摘要截短至400字)