Bussel J B, Haimi J S
Department of Pediatrics, New York Hospital--Cornell Medical Center, NY 10021.
Am J Hematol. 1988 Jun;28(2):79-84. doi: 10.1002/ajh.2830280203.
Isolated thrombocytopenia occurs frequently in patients infected with HIV. Studies of mechanisms of thrombocytopenia and clinical response to therapy suggest that the thrombocytopenia is often antibody mediated (ITP). The best approach to treatment of these patients is uncertain in that the routine modalities (steroids, splenectomy, vinca alkaloids) that are used to increase the platelet count in patients with classic ITP are known to be immunosuppressive. We report here the results of intravenous gammaglobulin (IVGG) treatment of 22 patients with HIV-related acute and chronic ITP who had severe thrombocytopenia and bleeding symptoms. Only one patient had an opportunistic infection at the time of treatment. Eight patients were homosexual, eight had hemophilia, three were i.v. drug abusers, two children had congenital acquisition of HIV, and one was the wife of an HIV + i.v. drug abuser. The average pretreatment platelet count was 22,000/microliter (hemophiliacs were treated at higher platelet counts than were the other patients), and the mean peak platelet count measured on days 5 to 8 was 182,000/microliter. Nineteen of 22 patients had peak platelet counts greater than 50,000/microliter following IVGG and 17/22 had peak counts greater than 100,000/microliter. After the initial infusions, all but three refractory patients could maintain adequate platelet counts with IVGG alone infused no more often than once every 2 weeks. The outcomes for the 22 patients after multiple maintenance IVGG infusions were remission, 5; stable without therapy, 1; maintenance, 13; and refractory, 3. The eight hemophiliacs with ITP responded better than did the eight homosexual ITP patients; their mean peak platelet count was 227,000/microliter versus 142,000/microliter in the homosexuals. In summary, patients with HIV-related ITP without opportunistic infections responded well to IVGG, with peak platelet counts comparable to those of ITP patients not infected with HIV. IVGG may be a useful therapy of ITP in HIV+ patients, since it appears to be less immunosuppressive than are conventional therapies, and none of the 22 HIV+ patients developed an opportunistic infection while receiving IVGG alone.
孤立性血小板减少症在感染HIV的患者中频繁出现。对血小板减少症机制及治疗临床反应的研究表明,血小板减少症通常由抗体介导(免疫性血小板减少症)。治疗这些患者的最佳方法尚不确定,因为用于提高经典免疫性血小板减少症患者血小板计数的常规方法(类固醇、脾切除术、长春花生物碱)已知具有免疫抑制作用。我们在此报告静脉注射丙种球蛋白(IVGG)治疗22例患有与HIV相关的急性和慢性免疫性血小板减少症且有严重血小板减少和出血症状患者的结果。治疗时仅有1例患者发生机会性感染。8例为同性恋者,8例患有血友病,3例为静脉注射毒品者,2例儿童先天性感染HIV,1例为HIV阳性静脉注射毒品者的妻子。治疗前平均血小板计数为22,000/微升(血友病患者治疗时的血小板计数高于其他患者),第5至8天测得的平均血小板计数峰值为182,000/微升。22例患者中有19例在接受IVGG治疗后血小板计数峰值大于50,000/微升,17/22例患者血小板计数峰值大于100,000/微升。初次输注后,除3例难治性患者外,所有患者仅通过每2周不超过1次的IVGG输注就能维持足够的血小板计数。22例患者在多次维持性IVGG输注后的结果为:缓解5例;无需治疗病情稳定1例;维持13例;难治3例。8例患有免疫性血小板减少症的血友病患者的反应优于8例同性恋免疫性血小板减少症患者;他们的平均血小板计数峰值为227,000/微升,而同性恋者为142,000/微升。总之,无机会性感染的与HIV相关的免疫性血小板减少症患者对IVGG反应良好,血小板计数峰值与未感染HIV的免疫性血小板减少症患者相当。IVGG可能是治疗HIV阳性患者免疫性血小板减少症的一种有用疗法,因为它似乎比传统疗法的免疫抑制作用小,并且22例HIV阳性患者在仅接受IVGG治疗时均未发生机会性感染。