Martínez-Cordero E, Vega-Avila E, Negrete-García M C, Zamudio-Cortés P, Aguilar-Frausto I
Laboratorio de Biologia Celular y Autoinmunidad, Instituto Nacional de Enfermedades Respiratorias, México.
Bol Med Hosp Infant Mex. 1991 Jul;48(7):463-8.
We studied 30 children with systemic lupus erythematosus in order to detect the clinical, pathological and serological findings associated with the development of thrombocytopenic purpura and hemolytic anemia. A group of 13 of the 30 children revealed hematological manifestations as the most prominent changes of SLE. Thrombocytopenic purpura and hemolytic anemia were the beginning manifestation of SLE in 11 children. Different causes, for these hematological changes such as hypersplenism, renal microangiopathy or drugs reactions were excluded by history and examination. Interestingly, other immunohematological manifestations including splenomegaly and lymphadenopathy were more frequent in children with thrombocytopenic purpura and hemolytic anemia, than in those patients without these hematological complications. Positive antiplatelet antibodies were found in 4/6 children with thrombocytopenia and 2/5 with hemolytic anemia. A relation of antiplatelet with anticardiolipin antibodies occurred in 4 patients; 3 of them in children with thrombocytopenic purpura and one with hemolytic anemia. Anti-dsDNA and anti-Sm antibodies were positive in almost all patients. Four children shown a transition from anti-dsDNA to anti-Sm antibodies or viceversa and all of them revealed a significant variation in the titer of these antibodies by ELISA, in relation with disease activity. The presence of hematological manifestations associated with anti-platelet and anti-cardiolipin antibodies in children with SLE support that different mechanisms triggers autoimmunity in childhood.
我们研究了30例系统性红斑狼疮患儿,以检测与血小板减少性紫癜和溶血性贫血发生相关的临床、病理和血清学表现。30例患儿中有13例表现出血液学表现,为系统性红斑狼疮最显著的变化。血小板减少性紫癜和溶血性贫血是11例患儿系统性红斑狼疮的首发表现。通过病史和检查排除了这些血液学变化的不同原因,如脾功能亢进、肾微血管病变或药物反应。有趣的是,与无这些血液学并发症的患儿相比,血小板减少性紫癜和溶血性贫血患儿出现脾肿大和淋巴结病等其他免疫血液学表现更为频繁。在6例血小板减少患儿中有4例、5例溶血性贫血患儿中有2例发现抗血小板抗体阳性。4例患者抗血小板抗体与抗心磷脂抗体有关;其中3例为血小板减少性紫癜患儿,1例为溶血性贫血患儿。几乎所有患者抗双链DNA和抗Sm抗体均为阳性。4例患儿出现抗双链DNA抗体向抗Sm抗体的转变或反之,并且通过酶联免疫吸附测定法(ELISA)发现,所有这些患儿这些抗体的滴度均随疾病活动度出现显著变化。系统性红斑狼疮患儿中存在与抗血小板和抗心磷脂抗体相关的血液学表现,这支持不同机制引发儿童自身免疫。