TY - JOUR
T1 - Inherited chromosomally integrated human herpesvirus-6 in a patient with XIAP deficiency
AU - Inoue, Kento
AU - Miura, Hiroki
AU - Hoshino, Akihiro
AU - Kamiya, Takahiro
AU - Tanita, Kay
AU - Ohye, Tamae
AU - Park, Myoung Ja
AU - Yanagimachi, Masakatsu
AU - Takagi, Masatoshi
AU - Imai, Kohsuke
AU - Morio, Tomohiro
AU - Yoshikawa, Tetsushi
AU - Kanegane, Hirokazu
N1 - Funding Information:
This work was supported by MEXT/JSPS KAKENHI Grant Number JP17K10099 to H. K. We thank the medical staff of Gunma Children's Medical Center and the Tokyo Medical and Dental University Hospital for taking care of the patient. We also thank Enago (www.egano.jp) for the English-language review.
PY - 2020/10/1
Y1 - 2020/10/1
N2 - Human herpesvirus-6 (HHV-6) is a common pathogen affecting the human population. Primary HHV-6 infection generally occurs during infancy and causes exanthema subitum. Moreover, HHV-6 may exhibit inherited chromosomally integrated HHV-6 (iciHHV-6) in certain individuals. Although iciHHV-6 is generally known to be nonpathogenic, it may cause reactivation in patients with primary immunodeficiency disease (PID). XIAP deficiency is a rare PID characterized by recurrent hemophagocytic lymphohistiocytosis (HLH). It has been reported that the Epstein-Barr virus primarily causes HLH; however, the other pathogens, including HHV-6, can also cause this complication. We encountered a case of XIAP deficiency accompanied by iciHHV-6. He suffered from recurrent HLH, for which allogeneic bone marrow transplantation (BMT) was performed as a curative therapy. During the course of BMT, the patient experienced HLH three times, but there was no reactivation of endogenous HHV-6 from iciHHV-6. Finally, the patient achieved complete donor chimerism and a decline in HHV-6 DNA copy number in whole blood. This case report demonstrates no evidence of reactivation of iciHHV-6 during BMT in a patient with XIAP deficiency.
AB - Human herpesvirus-6 (HHV-6) is a common pathogen affecting the human population. Primary HHV-6 infection generally occurs during infancy and causes exanthema subitum. Moreover, HHV-6 may exhibit inherited chromosomally integrated HHV-6 (iciHHV-6) in certain individuals. Although iciHHV-6 is generally known to be nonpathogenic, it may cause reactivation in patients with primary immunodeficiency disease (PID). XIAP deficiency is a rare PID characterized by recurrent hemophagocytic lymphohistiocytosis (HLH). It has been reported that the Epstein-Barr virus primarily causes HLH; however, the other pathogens, including HHV-6, can also cause this complication. We encountered a case of XIAP deficiency accompanied by iciHHV-6. He suffered from recurrent HLH, for which allogeneic bone marrow transplantation (BMT) was performed as a curative therapy. During the course of BMT, the patient experienced HLH three times, but there was no reactivation of endogenous HHV-6 from iciHHV-6. Finally, the patient achieved complete donor chimerism and a decline in HHV-6 DNA copy number in whole blood. This case report demonstrates no evidence of reactivation of iciHHV-6 during BMT in a patient with XIAP deficiency.
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U2 - 10.1111/tid.13331
DO - 10.1111/tid.13331
M3 - Article
C2 - 32424944
AN - SCOPUS:85086087077
VL - 22
JO - Transplant Infectious Disease
JF - Transplant Infectious Disease
SN - 1398-2273
IS - 5
M1 - e13331
ER -