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Pathology of Severe Fever with Thrombocytopenia Syndrome

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Abstract

Severe fever with thrombocytopenia syndrome (SFTS) is a novel viral infectious disease with high case fatality rate (CFR). SFTS is caused by a novel phlebovirus, SFTS virus (SFTSV). SFTS, a tick-borne viral infection, was reported to be endemic to central and northeastern China and is also endemic to South Korea and western Japan. Patients with SFTS show symptoms of fever, general fatigue, and gastrointestinal symptoms such as diarrhea, whereas those severely ill with SFTS usually show gastrointestinal hemorrhage and deteriorated consciousness. The CFR of SFTS patients ranges from 5 to 40%. Because the time from the discovery of SFTS is short, pathological studies on SFTS remain limited. However, the existing pathological studies revealed that SFTSV replicated mainly in the lymphoreticular organs such as lymph nodes, spleen, and liver. Furthermore, SFTSV antigen-positive cells showed multi-organ infiltration, including to central nervous cells, kidneys, and lungs. Virus infection-related hemophagocytic syndrome associated with cytokine storm is also seen in most severe SFTS patients, suggesting that the mechanisms behind the high CFR may be multi-organ failure induced by hemophagocytic syndrome and coagulopathy due to disseminated intravascular coagulation causing bleeding tendency. To reduce the morbidity and mortality of SFTS, understanding of the pathophysiology of SFTS is required.

Keywords

  • Severe fever with thrombocytopenia syndrome
  • SFTS
  • Pathology
  • Multiorgan failure
  • Hemophagocytosis
  • Cytokine storm

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Acknowledgements

The author acknowledges Drs. Toru Takahashi and Toshiaki Kamei, Yamaguchi Grand Medical Center, Yamaguchi, Japan, and Dr. Masahiko Kaneko, Department of Internal Medicine, Uwajima City Hospital, Ehime, Japan. The author also thanks Drs. Hideki Tani, Masayuki Shimojima, Shuetsu Fukushi, Tomoki Yoshikawa, and Takeshi Kurosu, Department of Virology 1, National Institute of Infectious Diseases (NIID); Drs. Hideki Hasegawa and Tadaki Suzuki, Department of Pathology, NIID; and Dr. Shigeru Morikawa, Department of Veterinary Medicine, NIID, Tokyo, Japan, for their contributions. The studies presented in this review article were performed with the support of a grant-in-aid from the Ministry of Health, Labor and Welfare, Japan (Grant-in-aid H25-Shinko-Shitei-009) and grants from the Japan Agency for Medical Research and Development (18fk0108002, 18fk0108072, 19fk0108081).

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Correspondence to Masayuki Saijo .

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Saijo, M. (2019). Pathology of Severe Fever with Thrombocytopenia Syndrome. In: Saijo, M. (eds) Severe Fever with Thrombocytopenia Syndrome. Springer, Singapore. https://doi.org/10.1007/978-981-13-9562-8_12

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