Stefanini M
Department of Medicine, Tufts University School of Medicine, Boston, Massachusetts.
Nouv Rev Fr Hematol (1978). 1990;32(2):129-35.
Clinical findings, course, complications, outcome, response to treatment and possible pathogenetic mechanisms were evaluated in 1,122 cases of idiopathic (immunological) thrombocytopenic purpura seen over a period of 30 years. Evaluation of clinical and laboratory findings suggests that there are at least two types of idiopathic thrombocytopenic purpura. One, "allergic," is acute in onset, often related to exposure to viral or bacterial infections, is associated with more severe bleeding manifestations in the early stages, is in most cases self-limited, and responds favorably to corticosteroids in the event of very severe bleeding. Antiplatelet factors disappear upon achieving a clinical and hematological remission. The other, "autoimmune," has slow onset of symptoms, chronic course, may respond to corticosteroids and/or immunosuppressive drugs and more often to splenectomy, and is refractory to all therapy in a significant number of cases. Antiplatelet factors remain present even after clinical remission. Both forms are relatively benign processes. However, autoimmune (chronic) cases may show serious hemorrhagic complications and evolve into other autoimmune syndromes and malignant processes.
对30年间所见的1122例特发性(免疫性)血小板减少性紫癜患者的临床症状、病程、并发症、转归、治疗反应及可能的发病机制进行了评估。临床和实验室检查结果评估表明,特发性血小板减少性紫癜至少有两种类型。一种为“过敏性”,起病急,常与病毒或细菌感染有关,早期出血表现更严重,多数情况下为自限性,在出血非常严重时对皮质类固醇反应良好。达到临床和血液学缓解后抗血小板因子消失。另一种为“自身免疫性”,症状起病缓慢,病程慢性,可能对皮质类固醇和/或免疫抑制药物有反应,更多时候对脾切除术有反应,且在相当多病例中对所有治疗均耐药。即使临床缓解后抗血小板因子仍存在。两种类型均为相对良性的过程。然而,自身免疫性(慢性)病例可能出现严重的出血并发症,并演变为其他自身免疫综合征和恶性病变。