【摘要】：目的:回顧性總結(jié)分析噬血細(xì)胞綜合征(Hemophagocytic Syndrome,HPS)患者的臨床特征,結(jié)合國內(nèi)外文獻(xiàn)進(jìn)行綜述及討論,旨在提高各級各專業(yè)臨床醫(yī)師對該病的認(rèn)識和診斷水平。方法:選取21例近5年(2011.1-2015.12)山西醫(yī)科大學(xué)第二醫(yī)院診治的HPS患者,總結(jié)其病歷資料(病因、臨床表現(xiàn)、實(shí)驗(yàn)室化驗(yàn)指標(biāo)、主要治療策略及轉(zhuǎn)歸),對可能影響該病預(yù)后的因素進(jìn)行初步探討。結(jié)果:患者總數(shù)21例,其中男性9例(42.86%),女性12例(57.14%),男女二者之比是3:4;總體年齡分布區(qū)間為[18-76]歲,中位年齡為43歲。21例HPS患者最常見的病因是感染(14例),其中以單純EBV感染最多見(10例);淋巴瘤及病因不明者分別為2例及5例。臨床表現(xiàn)主要為:發(fā)熱及脾大(19例),肺部感染(16例),肝大(12例),淋巴結(jié)腫大及漿膜腔積液分別為11例及10例;此外少見的有黃染、瘀點(diǎn)、瘀斑、皮疹,少數(shù)合并中樞神經(jīng)系統(tǒng)癥狀、心功能不全、腎功能不全及消化道出血。實(shí)驗(yàn)室檢查常見為:二系或三系血細(xì)胞減少(21例),20例行鐵蛋白(SF)檢查中有19例升高,肝功能損害(18例),骨髓噬血現(xiàn)象(17例),18例行NK細(xì)胞活性檢查中有13例NK細(xì)胞活性下降/缺失;高甘油三脂血癥(10例),低纖維蛋白原血癥及凝血功能異常各占14例,3例合并DIC;1例行sCD25檢查明顯升高。治療方法,積極控制原發(fā)病,主要治療是激素、依托泊苷(VP16)和/或環(huán)孢素(CSA)治療;選用CHOP方案化療的是淋巴瘤患者(2例);4例患者應(yīng)用大劑量甲強(qiáng)龍(500mg-1g/日)沖擊治療,1例血栓性血小板減少性紫癜(TTP)聯(lián)合使用血漿置換。轉(zhuǎn)歸方面,9例患者早期好轉(zhuǎn)出院,12例因病情加重死亡;死亡患者中,1例合并消化道出血,合并肺部感染及凝血功能異常各10例,3例合并DIC,5例臟器功能衰竭。比較早期好轉(zhuǎn)組與死亡組患者的基本情況,包括初診時(shí)的年齡、性別、主要并發(fā)癥及實(shí)驗(yàn)室指標(biāo),發(fā)現(xiàn)兩組在初診時(shí)差異有統(tǒng)計(jì)學(xué)意義的指標(biāo)為血紅蛋白、SF、白細(xì)胞(WBC)及血小板(Plt)計(jì)數(shù)(所有P0.05)。結(jié)論:感染及腫瘤為繼發(fā)性HPS常見病因。臨床特征主要為發(fā)熱、肝脾/淋巴結(jié)腫大、二系或三系血細(xì)胞減少、SF升高、NK細(xì)胞活性下降或缺失、骨髓噬血現(xiàn)象、低纖維蛋白原(FIB)血癥,合并肝功能損害、肺部感染、凝血功能異常者占多數(shù)。早期診斷并及時(shí)治療對改善疾病預(yù)后大有裨益。早期患者的WBC計(jì)數(shù)和Plt計(jì)數(shù)減少、貧血及SF明顯升高可能與預(yù)后不良有關(guān)。影響該病預(yù)后的獨(dú)立因素則是Plt計(jì)數(shù)減少。預(yù)后較差者多合并肝功能損害、凝血功能異常、肺部感染及臟器功能衰竭。
[Abstract]:Objective: to review and analyze the clinical features of hemophagocytic syndrome (Hemophagocytic Syndrome,HPS) patients, and to improve the understanding and diagnosis of hemophagocytic syndrome (Hemophagocytic Syndrome,HPS). Methods: 21 cases of HPS patients treated in the second Hospital of Shanxi Medical University in recent 5 years (2011.2015.12) were selected and their medical records (etiology, clinical manifestation, laboratory test index, main treatment strategy and outcome) were summarized. The factors that may affect the prognosis of the disease were preliminarily discussed. Results: there were 21 patients, including 9 males (42.86%) and 12 females (57.14%). The ratio of male to female was 3: 4. The overall age distribution was [18-76] years, with a median age of 43 years. The most common etiology of 21 patients with HPS was infection (14 cases), in which EBV infection was the most common (10 cases), lymphoma and unknown etiology were 2 cases and 5 cases, respectively. The main clinical manifestations were fever and splenomegaly (19 cases), pulmonary infection (16 cases), hepatomegaly (12 cases), lymphadenopathy and serous cavity effusion in 11 cases and 10 cases respectively. In addition, there are rare yellow stain, stasis spot, ecchymosis, rash, a few complicated with central nervous system symptoms, cardiac insufficiency, renal insufficiency and gastrointestinal bleeding. Laboratory tests were performed in 21 cases of second or third line hemopenia, 19 cases of elevated ferritin (SF), 18 cases of liver dysfunction, and 17 cases of hemophagocytosis of bone marrow. Among 18 cases of NK cell activity test, 13 cases of NK cell activity decreased / deleted; Hypertriglyceridemia (n = 10), hypofibrinogenemia (n = 14) and coagulation dysfunction (n = 14) were found in each group. SCD25 was significantly increased in 3 patients with DIC;1. The main treatment was hormone etoposide (VP16) and / or cyclosporine (CSA). Four patients were treated with high dose of methylenolone (500mg-1g/ day) and one patient with thrombotic thrombocytopenic purpura (TTP) was treated with plasma exchange. In terms of prognosis, 9 patients were discharged from hospital early and 12 died because of aggravation of the disease, among them, 1 case was complicated with gastrointestinal hemorrhage, 10 cases were complicated with pulmonary infection and abnormal coagulation function, and 3 cases were complicated with DIC,5 organ failure. The basic conditions of the patients in the early improvement group and the death group were compared, including age, sex, main complications and laboratory indexes at the time of first visit. The results showed that the difference between the two groups at the first visit was hemoglobin, SF,. WBC (WBC) and platelet (Plt) count (all P0.05). Conclusion: infection and tumor are the common etiology of secondary HPS. The main clinical features were fever, hepatosplenomegaly, decrease of blood cells in second or third line, increase of SF, decrease or absence of NK cell activity, hemophagocytosis of bone marrow, hypofibrinogen (FIB), liver function damage and pulmonary infection. The majority of patients had abnormal coagulation function. Early diagnosis and timely treatment are helpful to improve the prognosis of the disease. The decrease of WBC count and Plt count in early patients and the increase of anemia and SF may be related to poor prognosis. The independent factor influencing the prognosis of the disease was the reduction of Plt count. Poor prognosis was associated with liver dysfunction, abnormal coagulation, pulmonary infection and organ failure.
【學(xué)位授予單位】：山西醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2016
【分類號】：R55

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