Germline HAVCR2突变在患有/不患有溶血性淋巴组织细胞吞噬综合征（HLH）的皮下脂肪样T细胞淋巴瘤（SPTCL）患者中频繁检测到，但与表现不同的因素仍未确定。为评估SPTCL和/或HLH的临床变异和相关因素，在患有SPTCL或特发性HLH / HLH样全身性疾病（仅定义为没有继发性原因的HLH）的患者DNA中进行了HAVCR2外显子2的直接测序。随后，使用随机效应Meta分析和多元逻辑回归对包括现有和先前发布的报道HAVCR2突变在SPTCL及其患有/不患有HLH人群的系统评价和个体患者数据（IPD）水平的Meta分析进行了分析。在34名受试者中，28名SPTCL患者中的10名发展为HLH / HLH样全身性疾病。HAVCR2Y82C突变的六例表现为没有脂肪坏死的HLH。男性性别（p=0.03）和年龄。

Germline HAVCR2 mutations are frequently detected in subcutaneous panniculitis-like T-cell lymphoma (SPTCL) patients with/without hemophagocytic lymphohistiocytosis (HLH) but factors associated with variable manifestations remain undetermined. To evaluate clinical variations and associated factors in SPTCL and/or HLH with/without HAVCR2 mutations, we performed direct sequencing of HAVCR2 exon 2 using DNA from patients with SPTCL or idiopathic HLH/HLH-like systemic illnesses, defined by HLH alone without secondary causes. The systematic review and individual patient data (IPD) level meta-analysis which included the present and previously published studies reporting HAVCR2 mutations in SPTCL with/without HLH populations was subsequently conducted using random-effects meta-analysis and multivariate logistic regression. Among 34 patients enrolled, 10 of 28 SPTCL patients developed HLH/HLH-like systemic illnesses. Six cases with HAVCR2Y82C mutation manifested with HLH without panniculitis. Male gender (p=0.03) and age.