Abstract:Objective: To investigate the clinical features and treatment experience of children with tsutsugamushi disease complicated with hemophagocytic lymphohistiocytosis (HLH). Methods: The epidemiology, general data, clinical manifestations, laboratory results, treatment and outcome of 8 cases of tsutsugamushi disease with HLH in our hospital from January 2014 to December 2020 were analyzed retrospectively. Results: All 8 children with tsutsugamushi disease met the diagnostic criteria of HLH. Eight cases were accompanied by varying degrees of abnormal transaminase, abnormal myocardial zymogram, hypoproteinemia and the proportion of CD4+ decreased significantly; 5 cases were accompanied by lacunar effusion and 3 cases were accompanied by abnormal cerebrospinal fluid. 7 of the cases were given doxycycline anti infection, IVIG combined with glucocorticoid and other supportive treatment in the early stage. 1 of the cases was given dexamethasone + etoposide induction chemotherapy in the early stage, and doxycycline was added to anti infection after the diagnosis of tsutsugamushi disease. The indexes of peripheral blood, liver function, myocardial enzyme spectrum of 8 cases were significantly improved, the liver and spleen were shrunk, and hemophagocytosis disappeared. No doxycycline related adverse reactions or hematologic diseases were found in the short-term follow-up. Conclusion: HLH is a rare complication of tsutsugamushi disease, which can be associated with multiple organ injuries. Effective anti-infection and combined treatment with IVIG and glucocorticoid can effectively alleviate HLH while chemotherapy may be not necessary.
李鑫, 谭力, 李明, 吕瑜, 张婷. 恙虫病继发噬血细胞性淋巴组织细胞增多症8例临床分析[J]. 河北医学, 2022, 28(2): 314-318.
LI Xin, TAN Li, LI Ming, et al. Clinical Analysis of 8 Cases of Hemophagocytic Lymphohistiocytosis Secondary to Tsutsugamushi Disease. HeBei Med, 2022, 28(2): 314-318.
[1] Akagi T,Mukai T,Tsuji S,et al.Scrub typhus (tsutsugamushi disease) in a patient presenting with hemophagocytic syndrome[J].Immunol Med,2018,41(2):82~84. [2] 刘静,李家斌.135例恙虫病临床特征及器官损伤相关因素分析[J].安徽医科大学学报,2018,53(8):1311~1313. [3] 徐晓军,汤永民.2018年国际组织细胞协会关于依托泊苷和骨髓移植治疗噬血细胞性淋巴组织细胞增生症的专家共识解读[J].中华儿科杂志,2019,57(10):752~756. [4] Lin M,Huang A,Zheng X,et al.Misdiagnosis of scrub typhus complicated by hemophagocytic syndrome[J].BMC Pediatr,2019,19(1):102. [5] McCrindle BW,Rowley AH,Newburger JW,et al.Diagnosis,treatment,and long-term management of kawasaki disease:a scientific statement for health professionals from the american heart association[J].published correction appears in Circulation,2019,140(5):e181~e184. [6] Mahajan SK,Mahajan SK.Neuropsychiatric manifestations of scrub typhus[J].Neurosci Rural Pract,2017,8(3):421~426. [7] Henter JI,Samuelsson-Horne A,Arico M,et al.Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation[J].Blood,2002,100(7):2367~2373. [8] 李莉,李晶,郑跃杰,等.腺病毒相关性噬血细胞综合征3例临床分析并文献复习[J].中国实用儿科杂志,2019,34(12):1030~1033. [9] 柯钟灵,方昕,陈燕惠.儿童恙虫病继发噬血细胞综合征的临床特点[J].临床儿科杂志,2018,36(7):529~532. [10] 侯明霞,叶生亮,刘凤娟,等.IVIG对PMA诱导的人巨噬细胞吞噬功能的影响[J].中国输血杂志,2018,31(3):243~247. [11] Chapagain RH,Agrawal S,Pokharel S,et al.Clinico-laboratory profile,complications and therapeutic outcome of scrub typhus in children[J].Nepal Health Res Counc,2020,18(2):282~287. [12] Naoi T,Morita M,Kawakami T,et al.Hemophagocytic lymphohistiocytosis associated with scrub typhus:systematic review and comparison between pediatric and adult cases[J].Trop Med Infect Dis,2018,3(1):19.