8例成人噬血细胞综合征临床病历回顾及讨论

发布时间：2018-06-20 07:51

本文选题：噬血细胞综合征 + 临床资料特点　；参考：《山东大学》2017年硕士论文

【摘要】：目的:本研究课题在于探讨8例成人噬血细胞综合征(Hemophagocytic Syndrome,HPS)的临床特点、临床诊疗过程及预后,并结合相关文献复习认识噬血细胞综合征发生发展过程。方法:回顾性分析山东大学附属济南市中心医院血液科2014年1月至2017年1月初诊8例HPS患者的临床资料特点,具体包括:8名噬血细胞综合征患者的一般资料、家族遗传史、临床表现、实验室检查、病理组织结果、影像学检查,治疗方案选择及转归等方面资料,并进行分析讨论。结果:我院2014年1月至2017年1月初诊8例HPS患者,均为成年人。其中男性2例,女性6例,男:女=0.3:1。按一般年龄阶段可分(青年期)20-35岁2例,男性1例,女性1例。(成年期)35-60岁4例,男性1例,女性3例。(老年期)60-80岁2例,均为女性。发病年龄(25～71岁),平均年龄(50.71土 16.92岁)。8例(100%)患者初诊时均有发热,脾大7例(87.5%),淋巴结肿大8例(100%),皮疹1例(12.5%),黄疸1例(12.5%),胸腔积液2例(25%),腹腔积液1例(12.5%),盆腔积液2例(25%),心包积液1例(12.5%),肺部感染5例(62.5%)。两系或三系血细胞减少6例(75%),三系均减少2例,两系减少4例。部分活化凝血酶原时间延长5例(62.5%),纤维蛋白原1.5g/L 3例(37.5%)。谷丙转氨酶升高3例(37.5%),谷草转氨酶升高4例(500%)。总胆红素升高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%)。乳酸脱氢酶升高6例(75%),L-谷丙酰转移酶升高7例(85.7%)。血清铁蛋白升高 7 例(87.5%),其中 5 例(62.5%)2 000 μ g/L。EB 病毒DNA检测阴性8例(100%)。骨髓噬血细胞阳性8例(100%)。病因分析,1例(12.5%)为重症感染。2例(28.6%)可疑淋巴细胞增殖性肿瘤,1例为T淋巴细胞淋巴瘤,1例为B淋巴细胞淋巴瘤。3例(37.5%)诊断为自身免疫性疾病,其中1例为未分化结缔组织病,1例为成人斯蒂尔病,1例为系统性红斑狼疮。2例(28.6%)原因不明。8例HPS患者治疗过程,3例予以依托泊苷+地塞米松为基础的化疗(其中2例联合环孢素A)。2例予以抗细菌、真菌及病毒等对症治疗。1例予以激素和丙种球蛋白等对症支持治疗。1例患者(考虑B淋巴细胞淋巴瘤),给予CVP方案化疗及对症治疗。1例应用地塞米松、环孢素及丙种球蛋白治疗。8例患者经初次治疗,有4例好转出院,住院时间(19-66)天,其中3例系自身免疫相关的HPS患者,1例系不明病因的HPS患者,随访时间最常7个月。2例患者放弃治疗,自动出院。其余2例死亡,住院时间分别为7天、31天,死亡率(25%)。结论:1.HPS为罕见的严重疾病,若患者临床表现为反复发热、肝脾或淋巴结肿大,或伴有皮疹、黄疸、神经精神症状等,血常规、凝血常规、铁蛋白异常、骨髓检查发现噬血细胞时应尽快排除HPS可能,以明确诊断,及时治疗。2.继发性HPS常见致病因素为恶性肿瘤、感染及免疫性疾病,可因HPS临床表现掩盖原发疾病诊断,容易出现误诊或漏诊,早期鉴别诊断很重要。3.噬血细胞综合征合并中枢神经系统受累临床少见,早期鉴别诊断困难,相关影像学检查可能指导诊断。4.如果噬血细胞综合征患者血红蛋白、血小板、纤维蛋白原、乳酸脱氢酶等明显异常可能提示其预后不良,应早期干预,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.
【学位授予单位】：山东大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R55

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