Hemophagocytic Lymphohistiocytosis: an Unusual Complication of Orientia tsutsugamushi Disease (Scrub Typhus)

被引:13
作者
Basheer, Aneesh [1 ]
Padhi, Somanath [2 ]
Boopathy, Vinoth [3 ]
Mallick, Saumyaranjan [5 ]
Nair, Shashikala [4 ]
Varghese, Renu G'Boy [2 ]
Kanungo, Reba [4 ]
机构
[1] Pondicherry Inst Med Sci, Dept Gen Med, Pondicherry 605014, India
[2] Pondicherry Inst Med Sci, Dept Pathol, Pondicherry 605014, India
[3] Pondicherry Inst Med Sci, Dept Gastroenterol, Pondicherry 605014, India
[4] Pondicherry Inst Med Sci, Dept Microbiol, Pondicherry 605014, India
[5] All India Inst Med Sci, Dept Pathol, New Delhi 110029, India
关键词
ACUTE RESPIRATORY-DISTRESS; REAL-TIME PCR; CLINICAL-FEATURES; SPOTTED FEVERS; DIAGNOSIS; INFECTION;
D O I
10.4084/MJHID.2015.008
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Background: Hemophagocytic lymphohistiocytosis (HLH) is an uncommon, potentially fatal, hyperinflammatory syndrome that may rarely complicate the clinical course of Orientia tsutsugamushi disease (scrub typhus). Methods: Here we describe the clinicopathological features, laboratory parameters, management, and outcome of three adult patients (1 female, 2 males) with scrub typhus associated HLH from a tertiary center. A brief and concise review of international literature on the topic was also added. Results: All three patients satisfied the HLH-2004 diagnostic criteria; one had multi-organ dysfunction with very high ferritin level (>30,000 ng/ml), and all had a dramatic recovery following doxycyclin therapy. Literature review from January 1990 to March 2014 revealed that scrub typhus associated HLH were reported in 21 patients, mostly from the scrub endemic countries of the world. These included 11 females and 10 males with a mean age of 35 years (range; 8 months to 81 years). Fifteen of 17 patients (where data were available) had a favorable outcome following early serological diagnosis and initiation of definitive antibiotic therapy with (N=6) or without (N=9) immunosuppressive/immunomodulator therapy. Mutation analysis for primary HLH was performed in one patient only, and HLH-2004 protocol was used in two patients. Conclusion: We suggest that HLH should be considered in severe cases of scrub typhus especially if associated with cytopenia (s), liver dysfunction, and coagulation abnormalities. Further studies are required to understand whether an immunosuppressive and/or immunomodulator therapy could be beneficial in those patients who remain unresponsive to definitive antibiotic therapy.
引用
收藏
页数:9
相关论文
共 38 条
[1]   Development of a real-time PCR assay for the diagnosis of scrub typhus cases in India and evidence of the prevalence of new genotype of O-tsutsugamushi [J].
Bakshi, Diprabhanu ;
Singhal, Pradeep ;
Mahajan, Sanjay K. ;
Subramaniam, Prasanna ;
Tuteja, Urmil ;
Batra, Harsh Vardhan .
ACTA TROPICA, 2007, 104 (01) :63-71
[2]  
Batra HV, 2007, INDIAN J MED RES, V126, P101
[3]   Recent advances in the diagnosis and treatment of hemophagocytic lymphohistiocytosis [J].
Bode, Sebastian F. N. ;
Lehmberg, Kai ;
Maul-Pavicic, Andrea ;
Vraetz, Thomas ;
Janka, Gritta ;
zur Stadt, Udo ;
Ehl, Stephan .
ARTHRITIS RESEARCH & THERAPY, 2012, 14 (03)
[4]   DIAGNOSTIC-CRITERIA FOR SCRUB TYPHUS - PROBABILITY VALUES FOR IMMUNOFLUORESCENT ANTIBODY AND PROTEUS OXK AGGLUTININ TITERS [J].
BROWN, GW ;
SHIRAI, A ;
ROGERS, C ;
GROVES, MG .
AMERICAN JOURNAL OF TROPICAL MEDICINE AND HYGIENE, 1983, 32 (05) :1101-1107
[5]   Scrub typhus, acute respiratory distress, and hemophagocytic lymphohistiocytosis [J].
Cascio, Antonio ;
Correnti, Patrizia ;
Iaria, Chiara .
INTERNATIONAL JOURNAL OF INFECTIOUS DISEASES, 2013, 17 (08) :E662-E662
[6]   Haemophagocytic syndrome and rickettsial diseases [J].
Cascio, Antonio ;
Giordano, Salvatore ;
Dones, Piera ;
Venezia, Silvia ;
Iaria, Chiara ;
Ziino, Ottavio .
JOURNAL OF MEDICAL MICROBIOLOGY, 2011, 60 (04) :537-542
[7]   Scrub typhus-associated hemophagocytic syndrome [J].
Chen, YC ;
Chao, TY ;
Lin, JC .
INFECTION, 2000, 28 (03) :178-179
[8]  
Diwan A G, 2014, J Assoc Physicians India, V62, P58
[9]   Scrub typhus associated macrophage activation syndrome [J].
Gopal, Gopinath Kango ;
Anugrah, Chrispal ;
Boorugu, Harikishan .
TROPICAL DOCTOR, 2010, 40 (04) :249-250
[10]   Scrub typhus complicated by acute respiratory distress syndrome and acute liver failure: a case report from Northeast India [J].
Goswami, Dibyajyoti ;
Hing, Arlangki ;
Das, Ananta ;
Lyngdoh, Monaliza .
INTERNATIONAL JOURNAL OF INFECTIOUS DISEASES, 2013, 17 (08) :E644-E645