Karpatkin S, Nardi M A
Department of Medicine, New York University Medical Center, NY 10016.
J Lab Clin Med. 1988 Apr;111(4):441-8.
The immunologic platelet profile of 29 patients with hemophilia who had received multiple transfusions (14 had thrombocytopenia) was compared with profiles of 15 patients with classic autoimmune thrombocytopenia. Thrombocytopenic hemophiliacs who were seropositive for the human immunodeficiency virus (13 out of 14) had platelet-bound immunoglobulin G and C3C4 levels as well as circulating immune complexes that were 15.1-, 4.0-, and 2.4-fold greater, respectively, than normal control subjects' platelets and 3.4-, 2.6-, and 2.4-fold greater, respectively, than autoimmune thrombocytopenic patients' platelets. Hemophiliacs with normal platelet counts (nine out of 13 seropositive) had elevated values that were intermediate in level between those of thrombocytopenic hemophiliacs and those of classic autoimmune thrombocytopenic patients. The four seronegative hemophiliacs had normal values (except for one platelet-bound IgG measurement). An inverse correlation was noted between platelet count and platelet-bound IgG, r = -0.838, P less than 0.001. Serum antiplatelet reactivity (1:256 mean titer compared with control sera) resided predominantly in the 7S IgG fraction, and bound to autologous as well as homologous platelets at concentrations as low as 0.06 to 0.13 mg/ml. F(ab')2 fragments of the 7S IgG fraction inhibited binding of hemophilic IgG to normal platelets and bound to normal platelets at concentrations as low as 0.06 mg/ml. Antiplatelet IgG could be eluted from six of six hemophilic and eight of eight classic autoimmune thrombocytopenic patients.(ABSTRACT TRUNCATED AT 250 WORDS)
对29例接受多次输血的血友病患者(其中14例有血小板减少症)的免疫血小板谱与15例经典自身免疫性血小板减少症患者的免疫血小板谱进行了比较。人类免疫缺陷病毒血清阳性的血小板减少症血友病患者(14例中有13例),其血小板结合免疫球蛋白G和C3C4水平以及循环免疫复合物分别比正常对照受试者的血小板高15.1倍、4.0倍和2.4倍,分别比自身免疫性血小板减少症患者的血小板高3.4倍、2.6倍和2.4倍。血小板计数正常的血友病患者(13例血清阳性中有9例)其值升高,处于血小板减少症血友病患者和经典自身免疫性血小板减少症患者之间的中间水平。4例血清阴性的血友病患者的值正常(除一项血小板结合IgG测量值外)。血小板计数与血小板结合IgG之间呈负相关,r = -0.838,P小于0.001。血清抗血小板反应性(与对照血清相比平均滴度为1:256)主要存在于7S IgG组分中,并且在低至0.06至0.13 mg/ml的浓度下与自体以及同源血小板结合。7S IgG组分的F(ab')2片段抑制血友病IgG与正常血小板的结合,并在低至0.06 mg/ml的浓度下与正常血小板结合。抗血小板IgG可从6例血友病患者中的6例以及8例经典自身免疫性血小板减少症患者中的8例中洗脱出来。(摘要截短至250字)