Secondary haemophagocytic lymphohistiocytosis: Experience from the Uppsala University Hospital

Abstract

Background. Haemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by fever, hepatosplenomegaly, cytopenia, and progressive multiple-organ failure. HLH in adults is often secondary to autoimmune diseases, cancer, or infections in contrast to familial HLH. Treatment of secondary HLH is directed against the triggering disease in addition to immunosuppressive therapy, the latter commonly according to the HLH-2004 protocol.

Methods. We conducted a retrospective study to identify triggering diseases, disease-specific and immunosuppressive therapy administered, and prognosis in adult patients with secondary HLH. Patient data were collected from October 2010 to January 2015.

Results. Ten adult patients with secondary HLH were identified. Seven were men, and the median age at diagnosis was 62 years. Five cases were triggered by malignant disease and five by infection. The median patient fulfilled five of the eight HLH-2004 diagnostic criteria. All patients fulfilled the criteria fever, cytopenia, and ferritin >500 µg/L. Median time from hospital admission to HLH diagnosis was 20 days. Four patients received immunosuppressive therapy according to the HLH-2004 protocol. The prognosis was dismal, especially for the patients with malignancy-associated HLH, of whom all died.

Conclusion.HLH should be suspected in patients who present with fever, cytopenia, and ferritin >500 µg/L. Secondary HLH has a dismal prognosis. None of the patients with HLH triggered by malignancy survived. Achieving remission of the triggering disease seems to be important for a favourable outcome as, in all surviving patients, the haemophagocytic syndrome resolved after remission of the underlying infection.     

显著高铁蛋白血症与噬血细胞性淋巴组织细胞增多症的相互关系

目的: 探讨显著高铁蛋白血症(marked hyperferritinemia,MHF)与噬血细胞性淋巴组织细胞增多症(hemophagocytic lymphohistiocytosis,HLH)患者的临床特征以及相关性。方法: 回顾性收集北京大学人民医院2017年1月至2018年9月急诊及住院的MHF患者的临床资料,包括患者一般资料,症状体征,血常规、生化、出凝血检测、血清铁蛋白检查,以及自然杀伤(natural killer, NK)细胞活性、可溶性白介素(interleukin, IL)-2受体、骨髓检查等。按是否诊断为HLH分为HLH组和非HLH组,按随访3个月结局分为死亡组与存活组,分别对各组进行比较分析。结果: 123例MHF患者平均年龄为(44.2±17.4)岁,男女比例为1.3 ∶1;常见病因为血液肿瘤、风湿免疫性疾病、铁超载、HLH。随着铁蛋白水平升高,HLH患者比例增加,铁蛋白在10 000~19 999、20 000~29 999、30 000~39 999、40 000~49 999、50 000 μg/L以上时,HLH占比分别为28.8%、40.0%、54.5%、50.0%、50.0%。HLH共46例(37.4%), 继发于肿瘤15例、风湿免疫性疾病14例、感染性疾病2例,不明原因15例。HLH组与非HLH组比较,两组间在年龄、性别、发热、意识障碍、初始铁蛋白、最高铁蛋白、血细胞改变、谷丙转氨酶(alanine aminotransferase, ALT)、谷草转氨酶(aspartate aminotransferase, AST)、总胆红素(total bilirubin,TBIL)、直接胆红素(direct bilirubin DBIL)、甘油三酯(triglyceride, TG)方面差异无统计学意义(P>0.05), 出凝血检测除纤维蛋白原(fibrinogen, Fib)外,差异也无统计学意义(P>0.05),在死亡率方面两组间差异无统计学意义(P>0.05);而在肝、脾、淋巴结肿大,白蛋白(albumin, ALB),Fib方面两组间差异有统计学意义(P<0.05)。死亡组与存活组比较,两组间在年龄,性别,发热,肝、脾、淋巴结肿大,初始铁蛋白,最高铁蛋白,中性粒细胞(neutrophil, Neu),血红蛋白(hemoglobin, Hb),ALT,AST,ALB,TG方面差异无统计学意义(P>0.05),出凝血检测除凝血酶原时间(prothrombin time, PT)外,两组间差异无统计学意义(P>0.05), HLH所占比例两组间差异也无统计学意义(P>0.05),而在意识障碍、血小板计数(platelet, PLT)、PT、TBIL、DBIL方面两组间差异有统计学意义(P<0.05)。结论: 随着铁蛋白水平升高,HLH患者的比例随之增加,但是MHF对于HLH诊断不具有特异性。     

NK细胞活性测定在HLH患儿早期诊断中的作用研究

目的探讨噬血细胞性淋巴组织细胞增多症(hemophagocytic lymphohistiocytosis,HLH)患者外周血NK细胞活性水平及其在早期诊断中的作用。方法收集2008年3月-2011年12月确诊HLH病例23例,31名正常健康儿童作为对照,采用流式细胞术检测外周血NK细胞活性,并将确诊HLH患者的NK细胞活性与正常人NK细胞活性进行比较。同时分析HLH-2004诊断标准中的发热、脾大、血细胞减少、高三酰甘油血症、低纤维蛋白血症、SF水平、骨髓中发现噬血现象阳性率。结果 23例HLH患儿NK活性均明显低于正常对照组,两者相比差异有统计学意义(P<0.01),且发现NK细胞活性在早期100%出现降低。结论 NK细胞活性下降在HLH早期诊断中具有可靠的诊断价值。     

32例成人继发性噬血细胞综合征临床特点和预后分析

目的 探讨成人继发性噬血细胞综合征(HLH)临床特征和预后因素。 方法 收集32例成人继发性HLH患者临床资料,其中男17例(53%),女15 例(47%),19~88岁,中位年龄57岁。回顾性分析HLH患者的临床特点、病因及复发,采用单因素和Cox多因素分析预后因素。 结果 32例发热和血清铁蛋白升高,26例肝脾肿大,25例血细胞减少。病因分析中恶性肿瘤相关14例(43.75%),感染相关10例(31.25%),其他8例(25%)。32例最终死亡24例(75%),中位生存期27 d,复发率11.10%。单因素方差分析示,年龄、皮疹、血红蛋白、甘油三酯、凝血时间、活化部分凝血酶原时间与死亡相关(P<0.05),Cox多因素分析显示,年龄、甘油三酯水平是预后的独立危险因素。 结论 成人继发性HLH病因和临床特点多样,高龄和高甘油三酯是其死亡相关的独立预后因子。     

血源播散性肺结核继发噬血细胞综合征1例

Hemophagocytic lymphohistiocytosis (HLH) was a life-threatening syndrome due to the uncontrolled immune activation of cytotoxic T lymphocytes, natural killer (NK) cells, and macrophages. HLH is characterized by primary and secondary causes, the early diagnosis and treatment of patients are closely related to the prognosis and clinical outcome of patients. The clinical presentation is variable but mostly includes prolonged fever, splenomegaly, coagulopathy, hypertriglyceridemia, and hemophagocytosis, none of them is specific and particular for HLH. Tuberculosis (TB) infection is one of the causes of HLH. HLH caused by TB is very rare clinically, but it has a high mortality. For patients with fever of unknown origin, HLH-related clinical manifestations sometimes present before the final diagnosis of TB, and HLH is associated with the most significant mortality rate. This article is mainly about a 28-year-old patient with HLH who suffered from severe TB infection. The patient attended a hospital with a history of 2 months of prolonged fever, 10 days booger and subcutaneous hemorrhage in lower limbs. Before this, he was in good health and denied any history of tuberculosis exposure. Combined with relevant laboratory test results (such as splenomegaly, hemoglobin, platelet count, and hypertriglyceridemia) and clinical manifestations (e.g. fever), the patient was diagnosed with hemophagocytic lymphohistiocytosis, but the etiology of HLH remained to be determined. To confirm the etiology, the patient was asked about the relevant medical history (intermittent low back pain) and was performed chest CT scan, bone marrow biopsy, and fundus photography. Finally, he was diagnosed with hemophagocytic lymphohistiocytosis caused by hematogenous disseminated pulmonary tuberculosis. In response to this, intravenous methylprednisolone and anti-tuberculosis treatment (isoniazid, pyrazinamide, moxifloxacin, and amikacin) were administered to the patient. After more than a month of treatment, the patient recovered from HLH caused by severe TB infection. Therefore, this case suggests that we should be vigilant to the patient who admitted to the hospital with fever for unknown reasons, to diagnose HLH as early as possible and clarify its cause, then perform interventions and treatment, especially HLH secondary to tuberculosis. Also, cases of atypical TB and severe TB should be carefully monitored to achieve early diagnosis and early intervention.     

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。     

成人噬血细胞淋巴组织增生症30例临床分析

目的：探讨噬血细胞淋巴组织增生症（HLH）的病因及临床特点，提高对HLH的认识。方法：回顾性分析30例噬血细胞淋巴组织增生症患者的临床资料。结果：符合Imashuku继发性HLH修订标准的27例患者中，病因不明HLH8例，占30％，其中6例病情危重。肿瘤相关HLH10例，占37％，其中5例T／NK细胞淋巴瘤4例死亡。各种感染（包括曲霉菌、伤寒杆菌、EB病毒等）相关HLH5例，占19％，1例EB病毒感染者死亡。自身免疫性疾病相关HLH4例，占14％，免疫抑制治疗效果佳。噬血细胞占骨髓有核细胞〈2％组与≥2％组在病因构成、血小板数量等方面无显著差异。结论：成人HLH多为继发性，应尽可能明确病因，采取针对性治疗。危重患者宜尽早采用免疫化疗或尝试异基因造血干细胞移植。噬血细胞占骨髓有核细胞比例的高低不影响HLH的诊断。     

Clinical Features of Spontaneous Remission in the Classic Fever of Unknown Origin: A Retrospective Study

ObjectiveTo summarize the clinical features of spontaneous remission in classic fever of unknown origin (FUO).MethodsMedical records of 121 patients diagnosed with FUO at admission in Peking Union Medical College Hospital between January 2018 and June 2018 were reviewed retrospectively. Patients who were discharged without etiological diagnoses were followed for 2 years. The clinical features and outcomes of these patients were summarized. Multivariate logistic regression was used to analyze related factors of spontaneous remission of FUO.ResultsAfter excluding 2 patients who lost to follow-up, the etiology of 119 FUO patients were as follows: infectious diseases in 30 (25.2%) cases, connective tissue diseases in 28 (23.5%) cases, tumor diseases in 8 (6.7%) cases, other diseases in 6 (5.0%) cases, and unknown diagnoses in 47 (39.5%) cases. Totally, 41 patients experienced spontaneous remission of fever (the median time from onset to remission was 9 weeks, ranging from 4 to 39 weeks). In patients with spontaneous remission in FUO, lymphadenopathy was less common clinical manifestation, the levels of inflammatory markers including leukocyte count, neutrophil count, neutrophil ratio, C-reactive protein, and ferritin were lower, and the proportion of CD8 positive T lymphocytes expressing CD38 was lower. Multivariate logistic regression analysis of factors with a P-value < 0.05 in univariate analysis shown that white blood cell count (OR: 0.545, 95%CI: 0.306-0.971, P = 0.039), neutrophil count (OR: 2.074, 95%CI: 1.004-4.284, P = 0.049), and proportion of neutrophils (OR: 0.928, 95%CI: 0.871-0.990, P = 0.022) were independent significant factors associated with spontaneous remission in FUO.ConclusionsThis study suggested that most patients discharged with undiagnosed classic FUO would remit spontaneously. Thus, for patients with stable clinical conditions, follow-up and observation could be the best choice. Patients with lower level of some inflammatory factors may have a high likelihood of spontaneous remission in classic FUO.     

血清铁蛋白、叶酸检测在血液系统恶性肿瘤患者中的临床价值

目的：探讨血清铁蛋白（Serum ferritin ,SF）、叶酸在血液系统恶性肿瘤患者诊断及预后中的临床价值。方法：采用放免法测定97例血液系统恶性肿瘤患者化疗前、后铁蛋白、叶酸的水平,分别观察对照组、治疗前、治疗后缓解组、部分缓解组及未缓解组血清铁蛋白、叶酸结果,进行组间比较。观察并比较不同铁蛋白水平下疾病的缓解率。结果：所观察的97例患者病程的各阶段均存在不同程度的铁蛋白增高、叶酸减低。尤以治疗前及治疗后未缓解组明显;治疗后完全缓解组SF、叶酸均有明显变化,与治疗前均值相比（P＜0．01）,但仍未恢复正常水平;经治疗后部分缓解组SF、叶酸与正常对照组及治疗前均值比较均有统计学意义（P＜0．01）;同时治疗前SF小于600ng/ml的患者常规化疗缓解率明显高于大于600ng/ml的患者（P＜0．01）。结论：血清铁蛋白及叶酸可作为血液系统恶性肿瘤学的指标,其测定不仅有助于诊断与预后的判断,还能够初步进行疗效评价,指导化疗方案的选择及调整。     

3例成人斯蒂尔病并发噬血细胞综合征患者的临床特点分析并文献复习

目的：探讨成人斯蒂尔病（AOSD）并发噬血细胞综合征（HPS）患者的临床表现、实验室指标特点和治疗预后情况,为其临床诊治提供依据。方法：收集3例AOSD并发HPS患者的性别、年龄、发热峰值温度、皮疹、关节肿痛、肝脾淋巴结肿大及治疗和预后情况等临床数据以及外周血白细胞（WBC）数量、中性粒细胞绝对值（NE#）、淋巴细胞绝对值（LY#）、单核细胞绝对值（MO#）、红细胞（RBC）、血红蛋白（HGB）、血小板（PLT）、天门冬氨酸氨基转移酶（AST）、谷丙氨酸氨基转移酶（ALT）、谷氨酰转肽酶（GGT）、碱性磷酸酶（ALP）、胆碱酯酶（CHE）、血清铁蛋白（SF）、纤维蛋白原（FBG）及骨髓象等实验室数据,并对其进行回顾性分析。结果：一般情况,3例患者均为50岁以上女性,出现HPS的时间分别为AOSD发病后的1个月、1个月和5个月。临床表现,3例AOSD患者均以发热、皮疹和关节疼痛为首发症状,均无肝脾肿大,仅患者3在AOSD初期出现淋巴结肿大,经糖皮质激素治疗后发热、皮疹、关节肿痛及淋巴结肿大症状均好转。并发HPS后3例患者均再次首先出现发热症状,体温均在39℃以上,其中2例患者出现黄疸,出现HPS后均未发现肝脾及淋巴结肿大。实验室指标特点,血细胞减少和持续升高的SF为AOSD并发HPS的早期敏感指标,肝功能酶学及FBG水平的变化均滞后于血细胞和SF变化。AOSD并发HPS患者肝功能异常以ALT水平升高最为明显。血细胞减少早期行骨穿活检即可发现吞噬现象。结论：AOSD并发HPS患者的临床特点为不明原因的再发持续高热,实验室指标的特点为血细胞减少（尤其是血小板减少）、SF持续升高、重度肝损伤（以ALT水平升高为主）、纤维蛋白原减少和骨穿活检出现吞噬现象;AOSD并发HPS患者应早发现、早治疗,以降低其复发率及死亡率。     

Correlation of haemoglobin and red cell indices with serum ferritin in Indian women in second and third trimester of pregnancy

Background

Iron deficiency anaemia (IDA) is the most common cause of anaemia in pregnancy in Indians and is associated with increased risk of low birth-weight infants. Studies from developed countries recommend iron supplementation based on serum ferritin levels. However, screening by serum ferritin is not feasible in all cases in India. This study was undertaken to document haematological profile of pregnant Indian women.

Methods

We studied the correlation between second and third trimester ferritin concentration and haemoglobin (Hb) and red cell indices in 100 consecutive ANC cases to select the best haematologic characteristic to identify women who needed iron therapy. Hb and red cell indices, RBC count, mean corpuscular volume, mean corpuscular haemoglobin, mean corpuscular haemoglobin concentration, red cell distribution width were analysed and PBS studied to subtype anaemia if present.

Results

Proportion of iron deficiency anaemia in pregnancy was 34% and significant correlation was found between serum ferritin and RDW-CV% and TRBC. No correlation was found between ferritin levels and Hb, MCV, MCH and MCHC. Serum ferritin levels were <12 ng/mL in 30 out of 52 non-anaemic cases suggesting prevalence of sub-clinical iron deficiency in 58% cases. None of the red cell indices correlated with ferritin level in this group. Only TRBC showed some correlation with ferritin (r = −0.090, p > 0.05).

Conclusion

All pregnant women in India should continue to get iron supplements unlike what is recommended in the developed countries where iron supplementation is based on serum ferritin levels.     

35例噬血细胞综合征临床特征与预后因素分析

目的总结噬血细胞综合征患儿的临床特征,探讨其预后及死亡危险因素。方法回顾性分析2003年1月~2009年12月我院收治的35例噬血细胞综合征患儿的临床特点、实验室检查及治疗。并采用SPSS10.0软件进行多因素Logistic回归分析,探讨患儿死亡的危险因素。结果 35例患儿中20例与感染相关（57.1%）,尤其是EBV感染诱发。其他原因4例（11.4%）,原因不明11例（31.4%）。临床表现发热34例（97.1%）,肝肿大31例（88.6%）、脾脏肿大23例（65.7%）,淋巴结肿大14例（40.0%）。实验室检查表现为不同程度的血细胞减少,其中贫血31例（88.6%）,血小板降低27例（77.1%）,白细胞降低18例（51.4%）。肝功能异常（100%）,高三酰甘油血症13例（37.1%）,低纤维蛋白原血症14例（40.0%）,血清铁蛋白增高15例（42.8%）,骨髓涂片找到噬血细胞33例（94.3%）。预后危险因素分析显示Hb〈30g/L是预后不良的危险因素,病死率高。结论噬血细胞综合征病因复杂,临床表现多样,病死率较高,早期诊断、早期化疗可提高患儿的生存率。     

继发性嗜血性淋巴细胞增生症治疗进展

继发性嗜血性淋巴组织细胞增生症(SHLH)是一组单核/巨噬细胞系统异常活化导致的致死性疾病,多继发于感染、恶性肿瘤、风湿性疾病等。SHLH预后与原发病有很大的关系。在引入HLH-94及随后的HLH-04方案后,SHLH预后有了明显的改善。SHLH的治疗取决于原发病的性质和病情,严重者采用自体骨髓移植有望治愈。     

Long-term Prognosis of Patients with Acute non-ST-segment Elevation Myocardial Infarction undergoing Different Treatment Strategies

Background:

In cardiology, it is controversial whether different therapy strategies influence prognosis after acute coronary syndrome. We examined and compared the long-term outcomes of invasive and conservative strategies in patients with non-ST-segment elevation myocardial infarction (NSTEMI) and characterized the patients selected for an invasive approach.

Methods:

A total of 976 patients with acute NSTEMI were collected from December 2006 to October 2012 in the First Affiliated Hospital of Dalian Medical University Hospital. They are divided into conservative strategy (586 patients) and invasive strategy (390 patients) group. Unified follow-up questionnaire was performed by telephone contact (cut-off date was November, 2013). The long-term clinical events were analyzed and related to the different treatment strategies.

Results:

The median follow-up time was 29 months. Mortality was 28.7% (n = 168) in the conservative group and 2.1% (n = 8) in the invasive management at long-term clinical follow-up. The secondary endpoint (the composite endpoint) was 59.0% (n = 346) in the conservative group and 30.3% (n = 118) in the invasive management. Multivariate analysis showed that patients in the conservative group had higher all-cause mortality rates than those who had the invasive management (adjusted risk ratio [RR] = 7.795; 95% confidence interval [CI]: 3.796–16.006, P < 0.001), and the similar result was also seen in the secondary endpoint (adjusted RR = 2.102; 95% CI: 1.694–2.610, P < 0.001). In the subgroup analysis according to each Thrombolysis in Myocardial Infarction risk score (TRS), log-rank analysis showed lower mortality and secondary endpoint rates in the invasive group with the intermediate and high-risk patients (TRS 3–7).

Conclusions:

An invasive strategy could improve long-term outcomes for NSTEMI patients, especially for intermediate and high-risk ones (TRS 3–7).     

急性白血病血清铁蛋白水平的变化及其临床意义

目的探讨急性白血病(AL)患者血清铁蛋白水平的变化及其临床意义。方法检测45例AL初发期、30例AL完全缓解期(CCR)及15例AL复发期、15例难治性AL患者的血清铁蛋白水平,并进行对照比较。结果初发组、复发组及难治组血清铁蛋白水平明显高于缓解组(P<0.001),AL初发期及复发期与难治性AL血清铁蛋白水平差异无显著性(P>0.05)。结论AL患者初发期、复发期及难治性AL存在明显的血清铁蛋白水平的升高,检测观察AL者血清铁蛋白的变化有助于对病情及预后的判断。     

Lupus Myocarditis: A Case–Control Study from China

Background:

Myocarditis is an uncommon but serious manifestation of systemic lupus erythematosus (SLE). This study aimed to investigate clinical characteristics and outcomes of lupus myocarditis (LM) and to determine risk factors of LM in hospitalized Chinese patients with SLE.

Methods:

We conducted a retrospective case–control study. A total of 25 patients with LM from 2001 to 2012 were enrolled as the study group, and 100 patients with SLE but without LM were randomly pooled as the control group. Univariable analysis was performed using Chi-square tests for categorical variables, and the Student''s t-test or Mann–Whitney U-test was performed for continuous variables according to the normality.

Results:

LM presented as the initial manifestation of SLE in 7 patients (28%) and occurred mostly at earlier stages compared to the controls (20.88 ± 35.73 vs. 44.08 ± 61.56 months, P = 0.008). Twenty-one patients (84%) experienced episodes of symptomatic heart failure. Echocardiography showed that 23 patients (92%) had decreased left ventricular ejection fraction (<50%) and all patients had wall motion abnormalities. A high SLE Disease Activity Index was the independent risk factor in the development of LM (odds ratio = 1.322, P < 0.001). With aggressive immunosuppressive therapies, most patients achieved satisfactory outcome. The in-hospital mortality was not significantly higher in the LM group than in the controls (4% vs. 2%,P = 0.491).

Conclusions:

LM could result in cardiac dysfunction and even sudden death. High SLE disease activity might potentially predict the occurrence of LM at the early stage of SLE. Characteristic echocardiographic findings could confirm the diagnosis of LM. Early aggressive immunosuppressive therapy could improve the cardiac outcome of LM.     

Evans综合征17例临床分析

目的:探讨成人Evans综合征的临床特征、诊断及治疗。方法:对17例成人Evans综合征患者进行回顾性分析。结果:所有患者均接受激素加或不加静脉丙种免疫球蛋白作为初始治疗,13例患者治疗后达完全缓解(CR)和部分缓解(PR);4例对激素耐药,随后给予免疫抑制剂治疗,2例患者获得CR和PR。结论:Evans综合征病情反复,综合治疗可以提高Evans综合征的疗效。     

系统性红斑狼疮血液学异常及治疗后的变化

目的:探讨系统性红斑狼疮(SLE)病人血液学异常改变及临床特征。方法:对62例SLE患者血液学资料及应用糖皮质激素、免疫抑制剂治疗的效果进行回顾性分析。结果:血象异常者为53例,占82.5%,以二系以上减少为主,46例进行了骨髓象检查,发现骨髓增生活跃或明显活跃40例,增生减低6例,表现为增生性贫血或特发性血小板减少性骨髓象,47例诊断后做Coombs试验,9例阳性,血细胞减少者,用糖皮质激素及免疫抑制剂治疗,血象均有升高。结论:血液系统是SLE易损器官,SLE病人血液学异常较常见,其特点是血液学改变多样性,缺乏特异性,以二系以上减少常见,骨髓象表现增生活跃为主,对糖皮质激素及免疫抑制治疗有效。     

儿童EB病毒相关性噬血细胞综合征52例临床特点及预后危险因素分析

目的：分析EB病毒相关性噬血细胞性淋巴组织细胞增生症（Epstein?Barr virus?associated hemophagocytic lymphohistiocytosis,EBV?HLH）患儿的临床特点、转归及预后危险因素。方法：选取南京医科大学附属南京儿童医院2012年8月—2018年9月诊治的EBV?HLH患儿52例,对患儿临床资料进行回顾性分析探讨影响预后的危险因素。结果：52例临床表现主要为持续性不规则发热（100.0%）、肝脏肿大（96.2%）及脾脏肿大（80.8%）。实验室检查中,所有患儿血浆EBV?DNA均为阳性（100.0%）,其他主要表现有肝功能异常（100.0%）、铁蛋白升高（94.3%）及甘油三酯升高（91.4%）。52例中11例死亡（包括4例复发）,好转或痊愈33例,出院后失访8例。生存组与死亡组在发热天数（P=0.036）、血红蛋白（P < 0.001）、谷氨酰转肽酶（P=0.040）、白蛋白（P=0.012）、凝血酶原时间（P=0.006）及国际标准化比值（P=0.028）之间差异有统计学意义。发热天数>14 d（P=0.003）、血红蛋白减少（P=0.003）、谷氨酰转肽酶增高（P=0.047）、低白蛋白血症（P=0.043）和凝血酶原时间延长（P=0.013）是影响预后的不良因素,EB病毒再次活跃是复发的重要原因（P < 0.001）。使用HLH?1994（或HLH?2004）方案患儿存活率更高,但因病例数太少无统计学意义（P=0.565）。结论：发热天数>14 d、血红蛋白下降、谷氨酰转肽酶增高、白蛋白下降、凝血酶原时间延长是影响EBV?HLH患儿预后的不良因素,EB病毒再活跃患儿更易复发,预后极差。     

川崎病并发巨噬细胞活化综合征(附3例报告并文献分析)

目的了解川崎病并发巨噬细胞活化综合征(MAS)病儿的特点。方法总结我院2009年11月—2010年4月收治的3例川崎病并发MAS病儿的临床特点,并对文献报道17例川崎病并发MAS病儿进行分析。结果 20例川崎病并发MAS病儿,男14例,女6例;年龄为1~11岁,平均(5.35±3.66)岁;临床特征主要有持续发热、肝脾大、血细胞减少、凝血功能障碍、肝酶升高、高三酰甘油血症、低纤维蛋白原血症、血清铁蛋白增高,骨髓细胞学检查找到噬血细胞。联合应用糖皮质激素、大剂量丙种球蛋白及免疫抑制剂治疗有效,20例病儿除1例放弃治疗,其余均存活。结论 MAS是川崎病的一种严重并发症,病情凶险,早期合理的治疗可改善川崎病并发MAS病儿的预后。