成人噬血细胞综合征临床分析：46例报告及文献复习

目的分析噬血细胞综合征（HPS）的临床表现、实验室特点、治疗及预后。方法回顾性分析南方医院46例资料完整的成人HPS患者的病因、临床表现、实验室检查结果及转归。结果46例患者中明确诊断肿瘤相关HPS 19例，感染相关性HPS 11例，10例行PET-CT考虑恶性淋巴瘤可能，6例病因不明。HPS-2004诊断标准中各指标的符合率分别为：发热100%，铁蛋白升高100%，两系或三系血细胞减少93.48%，脾大91.30%，噬血现象84.78%，低纤维蛋白原血症67.39%，高甘油三脂血症54.05%。比较肿瘤、感染、病因不明HPS 3组患者初诊时各实验室指标，Ferr、β2MG水平差异有统计学意义（P<0.05）。比较存活组和死亡组初诊时各实验室指标，TG、LDH、Fbg水平两组差异有统计学意义（P<0.05）。结论继发性HPS与多种潜在疾病有关，多伴有EB病毒感染，明确病因中以恶性肿瘤，尤其是NK/T细胞淋巴瘤为主要诱因。在HPS-2004的诊断标准中，发热、铁蛋白升高、血细胞减少灵敏性较高，HPS预后凶险，病死率高，尽快明确诊断至关重要，应早期采取针对性治疗，争取短期内控制病情。

Clinical characteristics of hemophagocytic syndrome: analysis of 46 cases

Objective To analyze the clinical manifestations, laboratory findings, treatment and prognosis of patients withhemophagocytic syndrome (HPS). Methods A retrospective study was carried out to analyze the underlying disease, clinicalcharacteristics, laboratory findings and outcomes of 46 patients with HPS. Results This cohort included 19 cases of HPSsecondary to cancer, 11 cases of HPS secondary to infection, 10 cases of suspected malignant lymphoma based on PET-CTfindings (without biopsy), and 6 cases of unknown etiology. The coincidence rate of the clinical characteristics of the patientswith the indices listed in HPS-2004 criteria were: fever (100% ), elevated serum ferritin (100% ), cytopenias (93.48% ),splenomegaly (91.30% ), hemophagocytosis in the bone marrow, spleen or lymph nodes (84.78% ), hypofibrinogenemia(67.39% ), and hypertriglyceridemia (54.05% ). The cases of cancer, infections and unknown etiology showed significantdifferences in serum levels of ferritin and β2MG (P<0.05), and significant differences were found in triglycerides, LDH, andfibrinogenemia between the nonfatal and fatal cases (P<0.05). Conclusion HPS can be secondary to various underlyingdiseases, many associated with Epstein-Barr virus infection. Cancer, especially NK/T-cell lymphoma, is the main cause of HPS.Persistent fever, elevated serum ferritin level and cytopenias are the most sensitive indicators for diagnosis of HPS, and earlydiagnosis and treatment are critical to lower the mortality rate of this disease.