Warzynski M J, Barbour S D, Winkler-Pickett R T, Ortaldo J R
West Michigan Flow Cytometry Service, Ferguson Hospital, Grand Rapids 49503.
Cytometry. 1994 Dec 15;18(4):231-6. doi: 10.1002/cyto.990180408.
We describe two brothers with hemophilia and AIDS, with an unusually large percentage of CD3+, CD56+ lymphocytes. They experienced no major complications with opportunistic infections (OI) and infrequent secondary infections, even though they had nearly 0% CD4 lymphocytes for 3 years. Both patients died in 1991 of progressive cardiomyopathy. The patients' lymphocytes were immunophenotyped by flow cytometry and analyzed for functional cytolytic activity against K562 and HIV infected HUT 78 cell lines. Single color CD4 counts were 2-9% for 4 years. Dual color CD4 counts at our laboratory demonstrated 0-1% CD4 for the last 6 months. When CD3+ lymphocytes were examined, both patient 1 and patient 2 demonstrated a significantly higher proportion and absolute number of CD3"bright"+, CD56+ double-positive cells, 47% and 22%, respectively, compared to other HIV-positive children with hemophilia (< or = 2%). Functional studies with the K562 target cell line demonstrated the highest natural killer (NK) lymphocyte activity in patient 1 that could not be augmented by in vitro addition of IL-2, whereas patient 2 had no NK activity unless IL-2 was added. Functional studies with HIV-infected HUT-78 cells demonstrated patient 2 had cytolytic activity against HIV-infected cells and patient 1 had high nonspecific cytolytic activity even against uninfected HUT-78 cells, whereas controls had minimal activity to HUT-78 cells or HIV-infected HUT-78 cells. The case report raises a speculative question requiring a larger database, whether the anti-HIV activity and/or unusual clinical course without typical O.I. of some AIDS patients may be related to the presence of CD3"bright"+, CD56+ lymphocytes of their immune system.
我们描述了两名患有血友病和艾滋病的兄弟，他们的CD3⁺、CD56⁺淋巴细胞比例异常高。尽管他们连续3年CD4淋巴细胞几乎为0%，但未出现机会性感染（OI）的重大并发症，继发性感染也很少见。两名患者均于1991年死于进行性心肌病。通过流式细胞术对患者的淋巴细胞进行免疫表型分析，并分析其对K562和HIV感染的HUT 78细胞系的功能性细胞溶解活性。4年来单色CD4计数为2 - 9%。我们实验室的双色CD4计数显示，在过去6个月中CD4为0 - 1%。当检查CD3⁺淋巴细胞时，患者1和患者2的CD3“亮”⁺、CD56⁺双阳性细胞比例和绝对数量均显著高于其他患有血友病的HIV阳性儿童（≤2%），分别为47%和22%。对K562靶细胞系的功能研究表明，患者1的自然杀伤（NK）淋巴细胞活性最高，体外添加IL - 2也无法增强，而患者2除非添加IL - 2否则没有NK活性。对HIV感染的HUT - 78细胞的功能研究表明，患者2对HIV感染细胞具有细胞溶解活性，患者1即使对未感染的HUT - 78细胞也具有高非特异性细胞溶解活性，而对照组对HUT - 78细胞或HIV感染的HUT - 78细胞的活性极小。该病例报告提出了一个需要更大数据库来探讨的推测性问题，即一些艾滋病患者的抗HIV活性和/或无典型机会性感染的异常临床病程是否可能与其免疫系统中CD3“亮”⁺、CD56⁺淋巴细胞的存在有关。
