妊娠相关噬血细胞综合征6例临床分析
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摘要
目的分析观察妊娠相关噬血细胞综合征(HPS)的临床特点,提高对其认识和诊疗水平。方法收集2013年1月—2017年12月首都医科大学附属北京佑安医院肝病免疫科收治的HPS患者6例,对其病因、临床表现、实验室检查指标、治疗方案及临床转归进行回顾性分析。结果6例妊娠相关HPS患者中3例为初次妊娠,3例为再次妊娠。1例患者发生于妊娠早期,2例患者发生于产褥期。6例患者原发病以感染为主要原因,2例为病毒感染,2例为细菌感染,1例为成人Still病,1例病因不明。主要临床表现以持续高热、脾肿大最多见。所有患者均有sCD25的显著升高和NK细胞活性的下降,除血细胞减少外,肝功能损害、高三酰甘油血症、铁蛋白升高在妊娠相关HPS患者中的发生率较高;6例患者均获得长期生存并仍在随访中。结论妊娠期及产褥期存在异常的免疫调节,容易诱发炎性反应,继发HPS,对于妊娠期及产褥期出现持续发热、肝功能异常、血细胞减少的患者,需考虑到HPS可能,应积极检测相关指标,及时正确的诊断和治疗可获得良好的预后。
Objective To observe the clinical characteristics of pregnancy related hemophagocytic syndrome(HPS)and improve the level of understanding and diagnosis of HPS. Methods A retrospective analysis of the causes, clinical manifestations,laboratory tests, treatment schemes and clinical outcomes of 6 patients with HPS was performed. Results Of the6 patients with pregnancy related HPS, 3 were again pregnant, and the other 3 were the first pregnancy. 1 case occurred in the early pregnancy, and 2 cases occurred in the puerperium. All 6 cases were mainly caused by infection, 2 cases with virus infection, 2 cases with bacterial infection, 1 case with adult Still, and 1 case with unknown cause. The main clinical manifestation was persistent high fever(100%). All patients had significantly elevated sCD25 and decreased NK cell activity. Besides blood cell depletion,liver dysfunction,hypertriglyceridemia,and elevated ferritin were associated with a high incidence in pregnancy related HPS patients. All 6 patients received long term survival and were still followed up. Conclusion There is an abnormal immune regulation during pregnancy and puerperium. It is easy to induce inflammatory response and secondary HPS.For patients with persistent fever, abnormal liver function, and hemocyte reduction in pregnancy and puerperium, HPS should be taken into consideration, and the timely and correct diagnosis and treatment can obtain good prognosis.
Objective To observe the clinical characteristics of pregnancy related hemophagocytic syndrome(HPS)and improve the level of understanding and diagnosis of HPS. Methods A retrospective analysis of the causes, clinical manifestations,laboratory tests, treatment schemes and clinical outcomes of 6 patients with HPS was performed. Results Of the6 patients with pregnancy related HPS, 3 were again pregnant, and the other 3 were the first pregnancy. 1 case occurred in the early pregnancy, and 2 cases occurred in the puerperium. All 6 cases were mainly caused by infection, 2 cases with virus infection, 2 cases with bacterial infection, 1 case with adult Still, and 1 case with unknown cause. The main clinical manifestation was persistent high fever(100%). All patients had significantly elevated sCD25 and decreased NK cell activity. Besides blood cell depletion,liver dysfunction,hypertriglyceridemia,and elevated ferritin were associated with a high incidence in pregnancy related HPS patients. All 6 patients received long term survival and were still followed up. Conclusion There is an abnormal immune regulation during pregnancy and puerperium. It is easy to induce inflammatory response and secondary HPS.For patients with persistent fever, abnormal liver function, and hemocyte reduction in pregnancy and puerperium, HPS should be taken into consideration, and the timely and correct diagnosis and treatment can obtain good prognosis.
引文
[1]James WV,William JG.Hemophagocytic lymphohistiocytosis:diagnosis,pathophysiology,treatment,and future perspectives[J].Ann Med,2006,38(1):20-31.DOI:10.1080/07853890500465189.
[2]Shukla A,Kaur A,Hira HS.Pregnancy induced haemophagocytic syndrome[J].J Obstet Gynaecol India,2013,63(3):203-205.DOI:10.1007/s13224-011-0073-0.
[3]Teng CL,Hwang GY,Lee BJ,et al.Pregnancy-induced hemophagocytic lymphohistiocytosis combined with autoimmunehemolytic anemia[J].J Chin Med Assoc,2009,72(3):156-159.DOI:10.1016/S1726-4901(09)70043-7.
[4]Henter JI,Home A,AricóM,et al.HLH-2004:Diagnostic and therapeutic guideline for hemophagocytic lymphohistiocytosis[J].Pediatr Blood Cancer,2007,48(2):124-131.DOI:10.1002/pbc.21039.
[5]Roman LK,Gary JS.Secondary hemophagocytic lymphohistiocytosisin adults:an update on diagnosis and therapy[J].Clin AdvHematol Oncol 2012,10(11):726-732.
[6]王晶石,吴林,赵新颜,范丽娟,陈婧.肝功能异常为主要临床表现的噬血细胞综合征10例临床分析[J].临床肝胆病杂志,2011,27(2):189-191.
[7]Chmait RH,Meimin DL,Koo CH,Huffaker J.Hemophagocytic syndrome in pregnancy[J].Obstet Gynecol 2000,95(6 Pt 2):1022-1024.
[8]Osugi Y,Hara J,Tagawa S,et al.Cytokine production regulating Thl and Th2cytokines in hemophagocytic lymphohistiocytosis[J].Blood1997,89(11):41004103.
[9]Larroche C,Mouthon L.Pathogenesis of hemophagocyticsyndrome(HPS)[J].Autoimmun Rev 2004,3(2):69-75.DOI:10.1016/S1568-9972(03)00091-0.
[10]Rivière S,Galicier L,Coppo P,et al.Reactive hemophagocytic syndrome in adults:a retrospective analysis of 162 patients[J].Am J Med,2014,127(11):1118-1125.DOI:10.1016/j.amjmed.2014.04.034.
[11]Chien CT,Lee FJ,Luk HN,et al.Anesthetic management for cesarean delivery in a parturient with exacerbated hemophagocytic syndrome[J].Int J Obstet Anesth,2009,18(4):413-416.DOI:10.1016/j.ijoa.2009.02.016.
[12]Risdall RJ,McKenna RW,Nesbit ME,et al.Virus-associated hemophagocytic syndrome:a benign histiocyticproliferation distinct from malignant histiocytosis[J].Cancer.1979,44(3):993-1002.
[13]Rousselin A,Alavi Z,Le Moigne E,et al.Hemophagocytic syndrome in pregnancy:case report,diagnosis,treatment,and prognosis[J].Clin Case Rep,2017,5(11):1756-1764.DOI:10.1002/ccr3.1172.
[14]Tumian NR,Wong CL.Pregnancy-related hemophagocytic lymphohistiocytosis associated with cytomegalovirus infection:A diagnostic and therapeutic challenge[J].Taiwan J Obstet Gynecol,2015,54(4):432437.DOI:10.1016/j.tjog.2014.11.023.
[15]Arewa OP,Ajadi AA.Humanimmunodeficiency virus associated with haemophagocyticsyndrome in pregnancy:a case report[J].West Afr J Med,2011,30(1):66-68.
[16]Kaito K,Kobayashi M,Katayama T,et al.Prognostic factors ofhemophagocytic syndrome in adults:analysis of 34 cases[J].Eur J Haematol,1997,59(4):247-253.
[2]Shukla A,Kaur A,Hira HS.Pregnancy induced haemophagocytic syndrome[J].J Obstet Gynaecol India,2013,63(3):203-205.DOI:10.1007/s13224-011-0073-0.
[3]Teng CL,Hwang GY,Lee BJ,et al.Pregnancy-induced hemophagocytic lymphohistiocytosis combined with autoimmunehemolytic anemia[J].J Chin Med Assoc,2009,72(3):156-159.DOI:10.1016/S1726-4901(09)70043-7.
[4]Henter JI,Home A,AricóM,et al.HLH-2004:Diagnostic and therapeutic guideline for hemophagocytic lymphohistiocytosis[J].Pediatr Blood Cancer,2007,48(2):124-131.DOI:10.1002/pbc.21039.
[5]Roman LK,Gary JS.Secondary hemophagocytic lymphohistiocytosisin adults:an update on diagnosis and therapy[J].Clin AdvHematol Oncol 2012,10(11):726-732.
[6]王晶石,吴林,赵新颜,范丽娟,陈婧.肝功能异常为主要临床表现的噬血细胞综合征10例临床分析[J].临床肝胆病杂志,2011,27(2):189-191.
[7]Chmait RH,Meimin DL,Koo CH,Huffaker J.Hemophagocytic syndrome in pregnancy[J].Obstet Gynecol 2000,95(6 Pt 2):1022-1024.
[8]Osugi Y,Hara J,Tagawa S,et al.Cytokine production regulating Thl and Th2cytokines in hemophagocytic lymphohistiocytosis[J].Blood1997,89(11):41004103.
[9]Larroche C,Mouthon L.Pathogenesis of hemophagocyticsyndrome(HPS)[J].Autoimmun Rev 2004,3(2):69-75.DOI:10.1016/S1568-9972(03)00091-0.
[10]Rivière S,Galicier L,Coppo P,et al.Reactive hemophagocytic syndrome in adults:a retrospective analysis of 162 patients[J].Am J Med,2014,127(11):1118-1125.DOI:10.1016/j.amjmed.2014.04.034.
[11]Chien CT,Lee FJ,Luk HN,et al.Anesthetic management for cesarean delivery in a parturient with exacerbated hemophagocytic syndrome[J].Int J Obstet Anesth,2009,18(4):413-416.DOI:10.1016/j.ijoa.2009.02.016.
[12]Risdall RJ,McKenna RW,Nesbit ME,et al.Virus-associated hemophagocytic syndrome:a benign histiocyticproliferation distinct from malignant histiocytosis[J].Cancer.1979,44(3):993-1002.
[13]Rousselin A,Alavi Z,Le Moigne E,et al.Hemophagocytic syndrome in pregnancy:case report,diagnosis,treatment,and prognosis[J].Clin Case Rep,2017,5(11):1756-1764.DOI:10.1002/ccr3.1172.
[14]Tumian NR,Wong CL.Pregnancy-related hemophagocytic lymphohistiocytosis associated with cytomegalovirus infection:A diagnostic and therapeutic challenge[J].Taiwan J Obstet Gynecol,2015,54(4):432437.DOI:10.1016/j.tjog.2014.11.023.
[15]Arewa OP,Ajadi AA.Humanimmunodeficiency virus associated with haemophagocyticsyndrome in pregnancy:a case report[J].West Afr J Med,2011,30(1):66-68.
[16]Kaito K,Kobayashi M,Katayama T,et al.Prognostic factors ofhemophagocytic syndrome in adults:analysis of 34 cases[J].Eur J Haematol,1997,59(4):247-253.