自然杀伤（NK）细胞淋巴细胞增多症伴脾功能减退持续存在，未发展为白血病。
Persistence of Natural Killer (NK) cell lymphocytosis with hyposplenism without development of leukaemia.
作者信息
Khan Sujoy, Myers K
机构信息
Department of Haematology, Prince Charles Hospital, Merthyr Tydfil, Wales, CF47 9DT, UK.
出版信息
BMC Clin Pathol. 2005 Sep 7;5:8. doi: 10.1186/1472-6890-5-8.
BACKGROUND
Natural killer (NK) cell lymphocytosis usually has an indolent course and can progress into massive lymphocytosis with development of cytopenias and neoplastic diseases. NK-cells usually express one or more "NK-associated" antigens (CD16, CD56, CD57). Reactive expansions are seen in autoimmune diseases, viral infections, solid tumours and non-Hodgkin's lymphoma.
CASE PRESENTATION
We report a lady with a benign clinical course over 10 years and persistent CD8+/CD3-/CD57+/CD16+ LGL proliferation with presence of Howell-Jolly bodies (functional hyposplenism), an association not previously described.
CONCLUSION
We discuss the possible causes of clonal expansion and conclude that this may be part of the spectrum of immune dysregulation associated with NK-cell lymphocytosis.
背景
自然杀伤（NK）细胞淋巴细胞增多症通常病程进展缓慢，可发展为大量淋巴细胞增多，并伴有血细胞减少和肿瘤性疾病。NK细胞通常表达一种或多种“NK相关”抗原（CD16、CD56、CD57）。在自身免疫性疾病、病毒感染、实体瘤和非霍奇金淋巴瘤中可见反应性增生。
病例报告
我们报告一位女性患者，其临床病程长达10年，呈良性经过，存在持续的CD8+/CD3-/CD57+/CD16+大颗粒淋巴细胞增殖，并伴有豪-焦小体（功能性脾功能减退），这种关联此前未曾描述过。
结论
我们讨论了克隆性增生的可能原因，并得出结论，这可能是与NK细胞淋巴细胞增多症相关的免疫失调谱的一部分。
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