Koifman Julius, Granton John, Thenganatt John
Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
Division of Respirology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
Pulm Circ. 2016 Dec;6(4):614-615. doi: 10.1086/688490.
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive, life-threatening syndrome of excessive immune activation. Presentation is most common among the pediatric population, and cases in adults are rare. The number of nonhematologic presentations described in relation to HLH has been growing. We present a case involving a woman who developed HLH after autologous stem cell transplantation for mantle cell lymphoma. Months later, she received a diagnosis of pulmonary arterial hypertension (PAH) while undergoing treatment for her HLH. To our knowledge, PAH associated with adult HLH has only been described in the literature once before. PAH may now be a potential differential diagnosis for patients with HLH who present with respiratory symptoms.
噬血细胞性淋巴组织细胞增生症（HLH）是一种由过度免疫激活引起的侵袭性、危及生命的综合征。其症状在儿科人群中最为常见，成人病例罕见。与HLH相关的非血液学表现的病例数量一直在增加。我们报告一例套细胞淋巴瘤自体干细胞移植后发生HLH的女性病例。数月后，她在接受HLH治疗时被诊断为肺动脉高压（PAH）。据我们所知，成人HLH相关的PAH在文献中仅被描述过一次。PAH现在可能是出现呼吸道症状的HLH患者的一个潜在鉴别诊断。
