Espinosa Bautista Karla Adriana, Garciadiego Fossas Pamela, León Rodríguez Eucario
Centro Médico ABC, México D.F.
Gac Med Mex. 2013 Jul-Aug;149(4):431-7.
Hemophagocytic lymphohistiocytosis is a syndrome characterized by pathological immune activation that may occur as either a primary a familial disorder (associated with genetic mutations), or as a sporadic condition, associated to infections, malignancies or autoimmune diseases. The clinical picture is characterized by a disproportionate inflammation that causes fever, cytopenias, splenomegaly, bone marrow hemophagocytosis, hypertriglyceridemia and hypofibrinogenemia. Syndrome-related mortality is high, so it is important to maintain a high index of suspicion and start early treatment with immunochemotherapy and bone marrow transplantation in primary and refractory cases. In this article, we review the clinical manifestations, pathology, diagnosis and treatment of these patients.
噬血细胞性淋巴组织细胞增生症是一种以病理性免疫激活为特征的综合征，它可能作为原发性（家族性疾病，与基因突变有关）或散发性疾病出现，后者与感染、恶性肿瘤或自身免疫性疾病相关。临床表现的特征是炎症反应过度，导致发热、血细胞减少、脾肿大、骨髓噬血细胞现象、高甘油三酯血症和低纤维蛋白原血症。该综合征相关的死亡率很高，因此在原发性和难治性病例中，保持高度的怀疑指数并尽早开始免疫化学疗法和骨髓移植治疗非常重要。在本文中，我们回顾了这些患者的临床表现、病理学、诊断和治疗。
