Hemophagocytic syndrome after living donor liver transplantation: a case report with a review of the literature

Abstract Background Hemophagocytic syndrome (HPS) is a rare and potentially fatal complication following liver transplantation. Case presentation A 63-year-old woman with decompensated liver cirrhosis secondary to hepatitis B virus infection underwent living donor liver transplantation using the rig...

Full description

Bibliographic Details
Main Authors: Norifumi Iseda, Tomoharu Yoshizumi, Takeo Toshima, Akinari Morinaga, Takahiro Tomiyama, Junichi Takahashi, Takashi Motomura, Yohei Mano, Shinji Itoh, Noboru Harada, Toru Ikegami, Yuji Soejima
Format: Article
Language:English
Published: SpringerOpen 2018-08-01
Series:Surgical Case Reports
Subjects:
Online Access:http://link.springer.com/article/10.1186/s40792-018-0505-5
_version_ 1811315686164660224
author Norifumi Iseda
Tomoharu Yoshizumi
Takeo Toshima
Akinari Morinaga
Takahiro Tomiyama
Junichi Takahashi
Takashi Motomura
Yohei Mano
Shinji Itoh
Noboru Harada
Toru Ikegami
Yuji Soejima
author_facet Norifumi Iseda
Tomoharu Yoshizumi
Takeo Toshima
Akinari Morinaga
Takahiro Tomiyama
Junichi Takahashi
Takashi Motomura
Yohei Mano
Shinji Itoh
Noboru Harada
Toru Ikegami
Yuji Soejima
author_sort Norifumi Iseda
collection DOAJ
description Abstract Background Hemophagocytic syndrome (HPS) is a rare and potentially fatal complication following liver transplantation. Case presentation A 63-year-old woman with decompensated liver cirrhosis secondary to hepatitis B virus infection underwent living donor liver transplantation using the right posterior section of her husband’s liver (graft volume, 581 g; 56.8% of the recipient’s standard liver volume). She developed small-for-size syndrome on postoperative day (POD) 7, and HPS was diagnosed on POD 12 by bone marrow aspiration (white blood cells, 300/μL; neutrophils, 30/μL). Given that she tested negative for viral (hepatitis B virus and cytomegalovirus) and bacterial infections, it was considered likely to be secondary HPS. Steroid pulse therapy was initiated, and her white blood cell count increased to 4290/μL on POD 15, indicating that her peripheral blood leukocytes had improved. There were no surgical complications, but the patient died of prolonged graft dysfunction with bacterial sepsis on POD 14. Conclusions We report a rare case of HPS occurring 2 weeks after living donor liver transplantation with a right posterior section graft, diagnosed early via bone marrow aspiration. This clinical course implies an association between HPS and graft dysfunction such as small-for-size syndrome. Further studies of the mechanism of hypercytokinemia-induced HPS are required to confirm the optimal treatment for HPS.
first_indexed 2024-04-13T11:35:25Z
format Article
id doaj.art-a516d9d2c6f44b178f0ef3ec0a0704f1
institution Directory Open Access Journal
issn 2198-7793
language English
last_indexed 2024-04-13T11:35:25Z
publishDate 2018-08-01
publisher SpringerOpen
record_format Article
series Surgical Case Reports
spelling doaj.art-a516d9d2c6f44b178f0ef3ec0a0704f12022-12-22T02:48:28ZengSpringerOpenSurgical Case Reports2198-77932018-08-01411510.1186/s40792-018-0505-5Hemophagocytic syndrome after living donor liver transplantation: a case report with a review of the literatureNorifumi Iseda0Tomoharu Yoshizumi1Takeo Toshima2Akinari Morinaga3Takahiro Tomiyama4Junichi Takahashi5Takashi Motomura6Yohei Mano7Shinji Itoh8Noboru Harada9Toru Ikegami10Yuji Soejima11Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityAbstract Background Hemophagocytic syndrome (HPS) is a rare and potentially fatal complication following liver transplantation. Case presentation A 63-year-old woman with decompensated liver cirrhosis secondary to hepatitis B virus infection underwent living donor liver transplantation using the right posterior section of her husband’s liver (graft volume, 581 g; 56.8% of the recipient’s standard liver volume). She developed small-for-size syndrome on postoperative day (POD) 7, and HPS was diagnosed on POD 12 by bone marrow aspiration (white blood cells, 300/μL; neutrophils, 30/μL). Given that she tested negative for viral (hepatitis B virus and cytomegalovirus) and bacterial infections, it was considered likely to be secondary HPS. Steroid pulse therapy was initiated, and her white blood cell count increased to 4290/μL on POD 15, indicating that her peripheral blood leukocytes had improved. There were no surgical complications, but the patient died of prolonged graft dysfunction with bacterial sepsis on POD 14. Conclusions We report a rare case of HPS occurring 2 weeks after living donor liver transplantation with a right posterior section graft, diagnosed early via bone marrow aspiration. This clinical course implies an association between HPS and graft dysfunction such as small-for-size syndrome. Further studies of the mechanism of hypercytokinemia-induced HPS are required to confirm the optimal treatment for HPS.http://link.springer.com/article/10.1186/s40792-018-0505-5Hemophagocytic syndromeLiver transplantationSmall-for-size syndrome
spellingShingle Norifumi Iseda
Tomoharu Yoshizumi
Takeo Toshima
Akinari Morinaga
Takahiro Tomiyama
Junichi Takahashi
Takashi Motomura
Yohei Mano
Shinji Itoh
Noboru Harada
Toru Ikegami
Yuji Soejima
Hemophagocytic syndrome after living donor liver transplantation: a case report with a review of the literature
Surgical Case Reports
Hemophagocytic syndrome
Liver transplantation
Small-for-size syndrome
title Hemophagocytic syndrome after living donor liver transplantation: a case report with a review of the literature
title_full Hemophagocytic syndrome after living donor liver transplantation: a case report with a review of the literature
title_fullStr Hemophagocytic syndrome after living donor liver transplantation: a case report with a review of the literature
title_full_unstemmed Hemophagocytic syndrome after living donor liver transplantation: a case report with a review of the literature
title_short Hemophagocytic syndrome after living donor liver transplantation: a case report with a review of the literature
title_sort hemophagocytic syndrome after living donor liver transplantation a case report with a review of the literature
topic Hemophagocytic syndrome
Liver transplantation
Small-for-size syndrome
url http://link.springer.com/article/10.1186/s40792-018-0505-5
work_keys_str_mv AT norifumiiseda hemophagocyticsyndromeafterlivingdonorlivertransplantationacasereportwithareviewoftheliterature
AT tomoharuyoshizumi hemophagocyticsyndromeafterlivingdonorlivertransplantationacasereportwithareviewoftheliterature
AT takeotoshima hemophagocyticsyndromeafterlivingdonorlivertransplantationacasereportwithareviewoftheliterature
AT akinarimorinaga hemophagocyticsyndromeafterlivingdonorlivertransplantationacasereportwithareviewoftheliterature
AT takahirotomiyama hemophagocyticsyndromeafterlivingdonorlivertransplantationacasereportwithareviewoftheliterature
AT junichitakahashi hemophagocyticsyndromeafterlivingdonorlivertransplantationacasereportwithareviewoftheliterature
AT takashimotomura hemophagocyticsyndromeafterlivingdonorlivertransplantationacasereportwithareviewoftheliterature
AT yoheimano hemophagocyticsyndromeafterlivingdonorlivertransplantationacasereportwithareviewoftheliterature
AT shinjiitoh hemophagocyticsyndromeafterlivingdonorlivertransplantationacasereportwithareviewoftheliterature
AT noboruharada hemophagocyticsyndromeafterlivingdonorlivertransplantationacasereportwithareviewoftheliterature
AT toruikegami hemophagocyticsyndromeafterlivingdonorlivertransplantationacasereportwithareviewoftheliterature
AT yujisoejima hemophagocyticsyndromeafterlivingdonorlivertransplantationacasereportwithareviewoftheliterature