期刊文献+

噬血细胞性淋巴组织细胞增生症127例临床分析 被引量:7

Clinical analysis of 127 patients with hemophagocytic lymphohistiocytosis
下载PDF
导出
摘要 目的总结继发性噬血细胞性淋巴组织细胞增生症(hemophagocytic lymphohistiocytosis,HLH)患者的临床特点。方法回顾性分析北京协和医院2007年1月至2012年6月收治的127例继发性HLH患者的潜在疾病、临床特征、实验室检查结果及预后。结果 HLH患者的潜在疾病包括病毒感染(26例,20.5%)、结核感染(4例,3.1%)、自身免疫病(16例,12.6%)、血液系统恶性肿瘤(48例,37.8%)和原因不明者(33例,26.0%)。常见临床表现有发热(97.6%)、肝大(53.5%)、脾大(79.5%)、淋巴结肿大(55.1%)、消化道出血(13.4%)、肾损害(29.9%)及中枢神经系统损害(12.6%)。实验室检查可见血细胞减少(98.4%)、肝功能异常(97.6%)、低纤维蛋白原血症(67.5%)、高甘油三酯血症(85.2%)、高铁蛋白血症(97.5%)和骨髓涂片中的噬血现象(91.3%)。44例治愈的患者中,37例长期随访仍持续缓解。83例因潜在疾病进展、多器官功能衰竭或严重感染死亡。结论继发性HLH可见于多种病因,临床表现多样,患者病情往往较重、病死率高。起病年龄大、男性、脾大及合并弥散性血管内凝血(DIC)可能会影响HLH患者预后。 Objective To summarize the clinical features of patients with hemophagocytic lymphohistiocytosis(HLH)by analyzing the clinical features of 127 patients.Methods The underlying diseases,clinical characteristics,laboratory findings,responses to therapy as well as outcomes of the 127 patients with HLH were analyzed retrospectively.Results The underlying diseases included virus infection(n =26),tuberculosis infection(n =4),rheumatic diseases(n =16),heamatologic malignancies(n=48),and no causes could be identified(n=33).HLH was clinically characterized by high fever(97.6%),hepatomegaly(53.5%),splenomegaly(79.5%),lymphadenopathy(55.1%),gastrointestinal hemorrhage(13.4%),renal(29.9%)and central nervous system(12.6%)involvement.The major laboratory findings included cytopenia(98.4%),liver dysfunction(97.6%),hypofibrinogenemia(67.5%),hypertriglyceridemia(85.2%),serum ferritin(97.5%),and hemophagocytosis in bone marrow(91.3%).Forty-four patients recovered after treatment,and 37 patients archieved long-term remission during the follow-up.Eighty-three patients died because of the progression of the underlying diseases,multiple organ failure and severe infection.Conclusion There are various underlying diseases for HLH and there are many clinical characteristic manifestations for HLH.HLH is always a critical situation with high mortality.Late disease onset,male gender,splenomegaly and DIC may affect the outcome of HLH patients.
出处 《北京医学》 CAS 2013年第5期360-362,366,共4页 Beijing Medical Journal
关键词 噬血细胞性淋巴组织细胞增生症 潜在疾病 临床表现 预后 危险因素 Hemophagocytic lymphohistiocytosis(HLH)Underlying diseases Clinical manifestation Prognosis Risk factor
  • 引文网络
  • 相关文献

参考文献8

  • 1Freeman HR, Ramanan AV. Review of haemophagocytic lympho histiocytosis. Arch Dis Child,2011,96:688-693.
  • 2Janka GE. Familial and acquired hemophagocytic lymphohi stio-cytosis. Eur J Pediatr,2007,166:95-109.
  • 3Henter JI, Home A, Arico M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer,2007,48:124-131.
  • 4徐晓军,汤永民.噬血细胞综合征诊治研究进展[J].中华儿科杂志,2011,49(9):712-716. 被引量:24
  • 5Rouphael NG, Talati NJ, Vaughan C, et al. Infections associated with haemophagocytic syndrome. Lancet hffect Dis,2007,7:814- 822.
  • 6Atteritano M, David A, Bagnato G, et al. Haemophagocytie syn- drome in rheumatic patients. A systematic review. Eur Rev Med Pharmaeol Sci,2012,16:1414-1424.
  • 7孙桂珍,吉布强,李学亮,刘桂娟.成人噬血细胞综合征预后危险因素分析[J].中国现代医生,2012,50(13):145-146. 被引量:2
  • 8帅晓,刘霆,牛挺,等.中国西南地区单中心56例噬血细胞综合征回顾性临床分析.中国实验血液学杂志,2011,19:569-570.

二级参考文献6

共引文献24

同被引文献56

  • 1李甘地.成熟(外周)T细胞和NK细胞肿瘤(待续)[J].白血病.淋巴瘤,2004,13(6):368-371. 被引量:6
  • 2周立强,孙燕,谭文勇,李陶,王琦路,冯凤仪,王金万,储大同,石远凯,李晔雄,孙云田,吕宁.非霍奇金淋巴瘤1125例临床病理分析[J].癌症进展,2006,4(5):391-397. 被引量:47
  • 3陈捷,吴颖,田红.淋巴瘤相关性嗜血细胞综合征28例临床诊治分析[J].实用医学杂志,2007,23(17):2714-2716. 被引量:8
  • 4Janka GE, Lehmberg K .Hemophagocytic syndromes —an update[J]. Blood Rev, 2 014,28(4):135-142.
  • 5Henter JI, Horne AC, Arico M, ef al.HLH-2004: Diagnostic and therapeuticguidelines for hemophagocyticlymphohistiocytosisUl- Pediatr BloodCancer, 2007, 48(2):124-131.
  • 6Shipp MA. A predictive modelfor aggressive non-Hodgkin’slymphoma. The International Non-Hodgkin *s Lymphoma PrognosticFactors Project[J]. N Engl J Med, 1993,329(14):987-994.
  • 7Cheson BD, Pfistner B, Juweid ME,et a/. Revised response criteria formalignant lymphoma[J]. J Clin Oncol,2007, 25(5):579-586.
  • 8Lin N, Song Y, Zheng W, et a/.A prospective phase II study ofZ.-asparaginase-CHOP plus radiationin newly diagnosed extranodal NK/T-cell lymphoma, nasal typeL)]- JHematol Oncol, 201 3(6):44.
  • 9Yong W, Zheng W, Zhu J, et al./.-asparaginase in the treatment ofrefractory and relapsed extranodalNK/T-cell lymphoma, nasal type[J].Ann Hematol, 2009, 88(7):647-652.
  • 10Riviere S, Galicier L, Coppo P, et al.Reactive hemophagocytic syndromein adults: A multicenter retrospectiveanalysis of 162 patients[J]. Am J Med,2014,127(1 1):1 1 18-1 125.

引证文献7

二级引证文献20

;
使用帮助 返回顶部