本文選題：噬血細胞綜合征 + 臨床資料特點��； 參考：《山東大學》2017年碩士論文

【摘要】：目的:本研究課題在于探討8例成人噬血細胞綜合征(Hemophagocytic Syndrome,HPS)的臨床特點、臨床診療過程及預后,并結合相關文獻復習認識噬血細胞綜合征發(fā)生發(fā)展過程。方法:回顧性分析山東大學附屬濟南市中心醫(yī)院血液科2014年1月至2017年1月初診8例HPS患者的臨床資料特點,具體包括:8名噬血細胞綜合征患者的一般資料、家族遺傳史、臨床表現(xiàn)、實驗室檢查、病理組織結果、影像學檢查,治療方案選擇及轉歸等方面資料,并進行分析討論。結果:我院2014年1月至2017年1月初診8例HPS患者,均為成年人。其中男性2例,女性6例,男:女=0.3:1。按一般年齡階段可分(青年期)20-35歲2例,男性1例,女性1例。(成年期)35-60歲4例,男性1例,女性3例。(老年期)60-80歲2例,均為女性。發(fā)病年齡(25～71歲),平均年齡(50.71土 16.92歲)。8例(100%)患者初診時均有發(fā)熱,脾大7例(87.5%),淋巴結腫大8例(100%),皮疹1例(12.5%),黃疸1例(12.5%),胸腔積液2例(25%),腹腔積液1例(12.5%),盆腔積液2例(25%),心包積液1例(12.5%),肺部感染5例(62.5%)。兩系或三系血細胞減少6例(75%),三系均減少2例,兩系減少4例。部分活化凝血酶原時間延長5例(62.5%),纖維蛋白原1.5g/L 3例(37.5%)。谷丙轉氨酶升高3例(37.5%),谷草轉氨酶升高4例(500%)�？偰懠t素升高1例(12.5%)。白蛋白降低8例(100%)。甘油三酯升高6例(75%),其中≥3 mmol/L 4例(50%)。乳酸脫氫酶升高6例(75%),L-谷丙酰轉移酶升高7例(85.7%)。血清鐵蛋白升高 7 例(87.5%),其中 5 例(62.5%)2 000 μ g/L。EB 病毒DNA檢測陰性8例(100%)。骨髓噬血細胞陽性8例(100%)。病因分析,1例(12.5%)為重癥感染。2例(28.6%)可疑淋巴細胞增殖性腫瘤,1例為T淋巴細胞淋巴瘤,1例為B淋巴細胞淋巴瘤。3例(37.5%)診斷為自身免疫性疾病,其中1例為未分化結締組織病,1例為成人斯蒂爾病,1例為系統(tǒng)性紅斑狼瘡。2例(28.6%)原因不明。8例HPS患者治療過程,3例予以依托泊苷+地塞米松為基礎的化療(其中2例聯(lián)合環(huán)孢素A)。2例予以抗細菌、真菌及病毒等對癥治療。1例予以激素和丙種球蛋白等對癥支持治療。1例患者(考慮B淋巴細胞淋巴瘤),給予CVP方案化療及對癥治療。1例應用地塞米松、環(huán)孢素及丙種球蛋白治療。8例患者經(jīng)初次治療,有4例好轉出院,住院時間(19-66)天,其中3例系自身免疫相關的HPS患者,1例系不明病因的HPS患者,隨訪時間最常7個月。2例患者放棄治療,自動出院。其余2例死亡,住院時間分別為7天、31天,死亡率(25%)。結論:1.HPS為罕見的嚴重疾病,若患者臨床表現(xiàn)為反復發(fā)熱、肝脾或淋巴結腫大,或伴有皮疹、黃疸、神經(jīng)精神癥狀等,血常規(guī)、凝血常規(guī)、鐵蛋白異常、骨髓檢查發(fā)現(xiàn)噬血細胞時應盡快排除HPS可能,以明確診斷,及時治療。2.繼發(fā)性HPS常見致病因素為惡性腫瘤、感染及免疫性疾病,可因HPS臨床表現(xiàn)掩蓋原發(fā)疾病診斷,容易出現(xiàn)誤診或漏診,早期鑒別診斷很重要。3.噬血細胞綜合征合并中樞神經(jīng)系統(tǒng)受累臨床少見,早期鑒別診斷困難,相關影像學檢查可能指導診斷。4.如果噬血細胞綜合征患者血紅蛋白、血小板、纖維蛋白原、乳酸脫氫酶等明顯異�？赡芴崾酒漕A后不良,應早期干預,提高生存率。
[Abstract]:Objective: This study is to explore the clinical features, clinical process and prognosis of 8 adult Hemophagocytic Syndrome (HPS), and to review the development process of hemophagocytic syndrome in combination with relevant literature. Methods: a retrospective analysis of the Department of Hematology, Ji'nan Center Hospital Affiliated to Shandong University from January 2014 to 2017 The clinical data of 8 patients with HPS were diagnosed at the beginning of January, including the general data of 8 hemophagocytic syndrome patients, family genetic history, clinical manifestation, laboratory examination, pathological tissue results, imaging examination, treatment options and prognosis. Results: our hospital was diagnosed from January 2014 to early January 2017 8. HPS patients were all adults, including 2 male and 6 female, male: female =0.3:1. at 20-35 years of age (Adolescence) 2 cases, male 1, female 1. (adult) 35-60 years and 4 cases, male 1 cases and 3 cases. (aged) 60-80 years 2 cases, age (25~71 years), average age (50.71 soil).8 cases There were 7 cases of fever, splenomegaly (87.5%), 8 cases of lymph node enlargement (100%), 1 cases of rash (12.5%), 1 cases of jaundice (12.5%), 2 cases of pleural effusion (25%), 1 cases of peritoneal effusion (12.5%), pelvic effusion in 2 cases (25%), pulmonary infection and blood cell reduction. Partial activation of prothrombin was extended in 5 cases (62.5%), fibrinogen 1.5g/L in 3 cases (37.5%). Alanine aminotransferase was elevated in 3 cases (37.5%), cereal transaminase was elevated in 4 cases (500%). Total bilirubin increased in 1 cases (12.5%). Albumin decreased in 8 cases (100%). Triglycerides increased in 6 cases (500%). The increase of lactate dehydrogenase (L-), L- glutamic acid Acyltransferase was elevated in 7 cases (85.7%). Serum ferritin was elevated in 7 cases (87.5%), of which 5 (62.5%) 2000 mu g/L.EB virus DNA was negative in 8 cases (100%). 8 cases (100%) were positive for bone marrow hemophagocytic positive. The cause analysis, 1 cases (12.5%) were.2 cases of severe infection, suspected lymphoblastic tumor, T lymphocyte lymphoma, and B lymphatic lymphatic cases. .3 cases (37.5%) were diagnosed as autoimmune diseases, including 1 cases of undifferentiated connective tissue disease, 1 cases of adult Steele's disease, 1 cases of systemic lupus erythematosus.2 cases (28.6%) of unexplained.8 HPS patients, and 3 cases of etoposide + dexamethasone based chemotherapy (of which 2 cases of cyclosporin A) were treated with anti bacteria. 4 cases of.1 patients (B lymphocyte lymphoma) were treated with glucocorticoid and gamma globulin in the treatment of.1 cases, and CVP regimen chemotherapy and symptomatic treatment of.1 cases were treated with dexamethasone, cyclosporin and gamma globulin were treated for the first treatment in.8 patients, 4 patients were discharged from hospital and hospitalized (19-66) days, of which 3 were hospitalized. There were 1 patients with autoimmune related HPS and 1 HPS patients with unknown etiology. The most frequent follow-up time was 7 months, and.2 patients were abandoned and discharged automatically. The remaining 2 cases died and the hospitalization time was 7 days, 31 days, and mortality (25%). Conclusion: 1.HPS is a rare serious disease, if the patient's clinical manifestation is recurrent fever, liver splenic or lymph node enlargement, or With skin rash, jaundice, neuropsychiatric symptoms, blood routine, coagulation routine, ferritin abnormal, bone marrow examination found that hemophagocytic cells should exclude HPS as soon as possible, to make a clear diagnosis, and to treat the common pathogenic factors of secondary HPS in.2. for malignant tumor, infection and immune disease, which may cover the diagnosis of primary disease because of the clinical manifestation of HPS, easy to appear. Misdiagnosis or missed diagnosis, early differential diagnosis is very important for.3. hemophagocytic syndrome complicated with central nervous system involvement. Early differential diagnosis is difficult. Related imaging examinations may guide the diagnosis of.4. if hemophagocytic hemophagocytic syndrome is hemoglobin, platelets, fibrinogen, lactate dehydrogenase, etc. It should be intervened early to improve the survival rate.
【學位授予單位】：山東大學
【學位級別】：碩士
【學位授予年份】：2017
【分類號】：R55

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