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以“蓝莓松饼宝宝”为表现的新生儿噬血细胞性淋巴组织细胞增生症

Hemophagocytic Lymphohistiocytosis in a Newborn Presenting as "Blueberry Muffin Baby".

作者信息

Larson Krista N, Gaitan Sergio R, Stahr Benjamin J, Morrell Dean S

机构信息

Department of Dermatology, School of Medicine, University of North Carolina, Chapel Hill, Chapel Hill, North Carolina.

Aurora Diagnostics GPA Laboratories, Greensboro Pathology, Greensboro, North Carolina.

出版信息

Pediatr Dermatol. 2017 May;34(3):e150-e151. doi: 10.1111/pde.13128.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome characterized by uncontrolled activation and proliferation of proinflammatory cytokines. Initial presentation commonly includes fever, hepatosplenomegaly, and pancytopenia; 6 to 65% of cases also have a concurrent cutaneous eruption. We present the case of a 6-day-old premature infant boy with congenital severe thrombocytopenia, anemia, and hepatosplenomegaly who presented with several cutaneous violaceous papules and nodules and was found to have HLH.

摘要

噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见的、危及生命的高炎症综合征,其特征为促炎细胞因子的不受控制的激活和增殖。初始表现通常包括发热、肝脾肿大和全血细胞减少;6%至65%的病例还同时有皮肤疹。我们报告一例6天大的早产男婴病例,该患儿患有先天性严重血小板减少、贫血和肝脾肿大,出现了数个皮肤紫蓝色丘疹和结节,被诊断为HLH。

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