Larson Krista N, Gaitan Sergio R, Stahr Benjamin J, Morrell Dean S
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.
Pediatr Dermatol. 2017 May;34(3):e150-e151. doi: 10.1111/pde.13128.
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.
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。