Severe cutaneous papillomavirus disease after haemopoietic stem-cell transplantation in patients with severe combined immune deficiency caused by common γc cytokine receptor subunit or JAK-3 deficiency
Severe cutaneous papillomavirus disease after haemopoietic stem-cell transplantation in patients with severe combined immune deficiency caused by common γc cytokine receptor subunit or JAK-3 deficiency
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DOI：
10.1016/s0140-6736(04)16457-x
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发表时间：
2004-06-19
期刊：
影响因子：
168.9
通讯作者：
Fischer, A
中科院分区：
文献类型：
--
作者：
Laffort, C;Le Deist, F;Fischer, A
造血干细胞移植是治疗严重联合免疫缺陷的救命方法。然而，这种治疗的长期随访很少。有证据表明，持续存在的部分免疫缺陷与重大感染有关，包括严重的人乳头瘤病毒（HPV）疾病。我们对来自一个中心的41例严重联合免疫缺陷患者的严重HPV疾病进行了回顾性分析，这些患者在造血干细胞移植后存活了10年或更长时间。41例患者中有9例有广泛的限于皮肤的慢性HPV疾病，中位发病时间为移植后8年。其中4例有典型的疣状表皮发育不良，这是一种罕见的遗传性皮肤病。这9例患者的移植特征、免疫状态和嵌合性与其他患者没有显著差异。9例HPV疾病患者有严重的联合免疫缺陷，与常见γ受体细胞因子亚基或Janus激酶-3 （JAK-3）缺乏症相关。相比之下，患有其他形式的严重联合免疫缺陷的患者没有任何HPV疾病的迹象。遗传原因是唯一被确定为严重联合免疫缺陷的易感因素，这表明自然杀伤细胞或角化细胞中的γ - mac/ jak -3依赖性信号可能在抗hpv免疫中起作用。
Haemopoietic stem-cell transplantation is a life-saving treatment for severe combined immune deficiency. However, there has been little long-term follow-up of this treatment. There is evidence for the persistance of partial immunodeficiency associated with significant infections, including severe human papillomavirus (HPV) disease. We did a retrospective analysis of severe HPV disease in a group of 41 patients with severe combined immune deficiency from one centre who were alive 10 years or longer after haemopoietic stem-cell transplantation. Nine of the 41 patients had extensive chronic HPV disease limited to the skin, with a median onset at 8 years after transplantation. Four had lesions typical of epidermodysplasia verruciformis, a rare genodermatosis. Transplant characteristics, immune status, and chimerism of these nine patients did not differ significantly from those of the other patients. The nine patients with HPV disease had severe combined immune deficiency associated with either common gammac receptor cytokine subunit or Janus kinase-3 (JAK-3) deficiency. By contrast, patients with other forms of severe combined immune deficiency did not have any signs of HPV disease. That genetic causes are the only predisposing factor to be identified for severe combined immune deficiency, suggests that natural-killer cells or gammac/JAK-3-dependent signalling in keratinocytes could have a role in anti-HPV immunity.