Bilateral lung transplantation in a patient with humoral immune deficiency: a case report with review of the literature.
Bilateral lung transplantation in a patient with humoral immune deficiency: a case report with review of the literature.
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DOI：
10.1155/2014/910215
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发表时间：
2014
影响因子：
1
通讯作者：
Walter JE
中科院分区：
文献类型：
--
作者：
Farmer JR;Sokol CL;Bonilla FA;Murali MR;Kradin RL;Astor TL;Walter JE
体液免疫缺陷与非感染性疾病并发症有关，包括自身免疫性血细胞减少症和肺部疾病。在此，我们提出了一个病人谁接受了脾切除术的自身免疫性血细胞减少症，随后被诊断为体液免疫缺陷的背景下，反复感染。在开始静脉注射免疫球蛋白（IVIG）治疗前进行的免疫球蛋白分析中，年龄匹配的伊加（11 mg/dL）、IgG 2（14 mg/L）和IgG 4（5 mg/L）血清水平较低，IgG总水平保持不变。流式细胞术对于B细胞成熟停滞在IgM+/IgD+阶段是显著的。对已知的原发性免疫缺陷引起的遗传缺陷进行选择性筛查，结果为阴性。病程因肺动静脉畸形（AVM）的发展而变得非常复杂，最终需要在2012年进行双侧肺移植。这是一个体液免疫缺陷的病人，在脾切除术后才变得明显，这就要求在接种疫苗和脾切除术前进行常规免疫学评估。肺移植是一种罕见的治疗终点，据我们所知，以前从未在体液免疫缺陷患者中描述过肺AVM适应症。
Humoral immune deficiencies have been associated with noninfectious disease complications including autoimmune cytopenias and pulmonary disease. Herein we present a patient who underwent splenectomy for autoimmune cytopenias and subsequently was diagnosed with humoral immune deficiency in the context of recurrent infections. Immunoglobulin analysis prior to initiation of intravenous immunoglobulin (IVIG) therapy was notable for low age-matched serum levels of IgA (11 mg/dL), IgG2 (14 mg/L), and IgG4 (5 mg/L) with a preserved total level of IgG. Flow cytometry was remarkable for B cell maturation arrest at the IgM+/IgD+ stage. Selective screening for known primary immune deficiency-causing genetic defects was negative. The disease course was uniquely complicated by the development of pulmonary arteriovenous malformations (AVMs), ultimately requiring bilateral lung transplantation in 2012. This is a patient with humoral immune deficiency that became apparent only after splenectomy, which argues for routine immunologic evaluation prior to vaccination and splenectomy. Lung transplantation is a rare therapeutic endpoint and to our knowledge has never before been described in a patient with humoral immune deficiency for the indication of pulmonary AVMs.