Acquired Hemophilia B Through Liver Transplantation: A Case Report and Literature Review

Christine Jane Kurian; Douglass Alan Drelich; Sanaa Rizk

doi:doi:10.11648/j.ajim.20200806.21

| Peer-Reviewed

Acquired Hemophilia B Through Liver Transplantation: A Case Report and Literature Review

Christine Jane Kurian, Douglass Alan Drelich, Sanaa Rizk

Published in American Journal of Internal Medicine (Volume 8, Issue 6)

Received: 4 October 2020 Accepted: 28 October 2020 Published: 16 December 2020

Views: Downloads:

Download PDF

Share This Article

Twitter
Linked In
Facebook

Abstract

Hemophilia B, also called Christmas disease, is an X-linked inherited bleeding disorder that predominantly affects males, and it is caused by deficiency of coagulation factor IX. Factor IX is a coagulation factor produced in the liver. Liver transplantation from hemophilia B donors is neither widely documented nor performed due to presumed risk of developing increased bleeding tendency in the recipient due to potential acquired factor IX deficiency. In this review, we present a case of liver and kidney transplantation from a donor with mild hemophilia B to a recipient with no history of hemophilia B and literature review. This is a relatively rare situation with only two other prior case reports of acquired Hemophilia B through liver transplantation noted in 2015. The 2 cases presented in the literature lead to acquired hemophilia B in liver transplant recipients since donors were not screened due to mild disease and low suspicion level. In our case, the donor was known to have mild Hemophilia B and the recipient had a rapid decline – thus it is imperative to weigh risks and benefits of transplantation from this donor population. While those individuals with mild hemophilia B disease (and low bleeding burden) should be considered as an alternative donor for liver transplantation, appropriate counseling should be done with the recipient about possible bleeding risks and OLT should only occur after patient consent. A multidisciplinary approach should involve hematology in case factor replacement therapy is needed post transplantation and for long term follow up.

Published in	American Journal of Internal Medicine (Volume 8, Issue 6)
DOI	10.11648/j.ajim.20200806.21
Page(s)	312-315
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Hemophilia B, Christmas Disease, Liver Transplant, Factor IX, Hemostasis

References

[1]	“F9 coagulation factor IX [Homo sapiens (human)] - Gene - NCBI.” Available at: https://www.ncbi.nlm.nih.gov/gene/2158. (Accessed: 24th February 2020).
[2]	P. H. B. Bolton-Maggs and K. J. Pasi, “Haemophilias A and B,” in Lancet, 2003, vol. 361, no. 9371, pp. 1801–1809, doi: 10.1016/S0140-6736(03)13405-8.
[3]	K. Kurachi and E. W. Davie, “Isolation and characterization of a cDNA coding for human factor IX,” Proc. Natl. Acad. Sci. U. S. A., vol. 79, no. 21 I, pp. 6461–6464, 1982, doi: 10.1073/pnas.79.21.6461.
[4]	K. A. Boost et al., “Long-term production of major coagulation factors and inhibitors by primary human hepatocytes in vitro: Perspectives for clinical application,” Liver Int., vol. 27, no. 6, pp. 832–844, Aug. 2007, doi: 10.1111/j.1478-3231.2007.01472.x.
[5]	S. Kasuda et al., “Expression of coagulation factors from murine induced pluripotent stem cell-derived liver cells,” Blood Coagul. Fibrinolysis, vol. 22, no. 4, pp. 271–279, Jun. 2011, doi: 10.1097/MBC.0b013e328344c63b.
[6]	K. Ohashi, K. Tatsumi, R. Utoh, S. Takagi, M. Shima, and T. Okano, “Engineering liver tissues under the kidney capsule site provides therapeutic effects to hemophilia B mice,” Cell Transplant., vol. 19, no. 6–7, pp. 807–813, Jun. 2010, doi: 10.3727/096368910X508924.
[7]	K. Tatsumi, K. Ohashi, M. Shima, Y. Nakajima, T. Okano, and A. Yoshioka, “Therapeutic effects of hepatocyte transplantation on hemophilia B,” Transplantation, vol. 86, no. 1, pp. 167–170, Jul. 2008, doi: 10.1097/TP.0b013e31817b9160.
[8]	K. Tatsumi et al., “Human Hepatocyte Propagation System in the Mouse Livers: Functional Maintenance of the Production of Coagulation and Anticoagulation Factors,” Cell Transplant., vol. 21, no. 2–3, pp. 437–445, Mar. 2012, doi: 10.3727/096368911X605349.
[9]	K. Bergstrom, A. Stevens, L. Srivaths, J. Economides, and D. L. Yee, “Haemophilia B acquired from liver transplantation: A case report and literature review,” Haemophilia, vol. 21, no. 4. Blackwell Publishing Ltd, pp. e328--e330, Jul-2015, doi: 10.1111/hae.12699.
[10]	D. M. Brunetta et al., “Hemophilia B acquired through liver transplantation,” Liver Transplantation, vol. 22, no. 2. John Wiley and Sons Ltd, pp. 254–256, Feb-2016, doi: 10.1002/lt.24364.
[11]	K. Tatsumi et al., “Hepatocyte is a Sole Cell Type Responsible for the Production of Coagulation Factor IX in Vivo,” Cell Med., vol. 3, no. 1–3, pp. 25–31, Jan. 2012, doi: 10.3727/215517912x639496.
[12]	D. Zanolini et al., “Extrahepatic sources of factor VIII potentially contribute to the coagulation cascade correcting the bleeding phenotype of mice with hemophilia A,” Haematologica, vol. 100, no. 7, pp. 881–892, Jul. 2015, doi: 10.3324/haematol.2014.123117.
[13]	C. L. Madeira et al., “Extrahepatic factor VIII production in transplant recipient of hemophilia donor liver.,” Blood, vol. 113, no. 21, pp. 5364–5365, May 2009, doi: 10.1182/blood-2009-02-206979.
[14]	T. Shahani et al., “Human liver sinusoidal endothelial cells but not hepatocytes contain factor VIII,” J. Thromb. Haemost., vol. 12, no. 1, pp. 36–42, Jan. 2014, doi: 10.1111/jth.12412.
[15]	C. J. Kurian, D. A. Drelich, and S. Rizk, “Successful liver transplant from a hemophilia A donor with no development of hemophilia A in recipient,” J. Thromb. Haemost., vol. 18, no. 4, pp. 853–856, Apr. 2020, doi: 10.1111/jth.14750.

Cite This Article

Plain Text BibTeX RIS

APA Style

Christine Jane Kurian, Douglass Alan Drelich, Sanaa Rizk. (2020). Acquired Hemophilia B Through Liver Transplantation: A Case Report and Literature Review. American Journal of Internal Medicine, 8(6), 312-315. https://doi.org/10.11648/j.ajim.20200806.21

Copy | Download

ACS Style

Christine Jane Kurian; Douglass Alan Drelich; Sanaa Rizk. Acquired Hemophilia B Through Liver Transplantation: A Case Report and Literature Review. Am. J. Intern. Med. 2020, 8(6), 312-315. doi: 10.11648/j.ajim.20200806.21

Copy | Download

AMA Style

Christine Jane Kurian, Douglass Alan Drelich, Sanaa Rizk. Acquired Hemophilia B Through Liver Transplantation: A Case Report and Literature Review. Am J Intern Med. 2020;8(6):312-315. doi: 10.11648/j.ajim.20200806.21

Copy | Download

@article{10.11648/j.ajim.20200806.21,
  author = {Christine Jane Kurian and Douglass Alan Drelich and Sanaa Rizk},
  title = {Acquired Hemophilia B Through Liver Transplantation: A Case Report and Literature Review},
  journal = {American Journal of Internal Medicine},
  volume = {8},
  number = {6},
  pages = {312-315},
  doi = {10.11648/j.ajim.20200806.21},
  url = {https://doi.org/10.11648/j.ajim.20200806.21},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajim.20200806.21},
  abstract = {Hemophilia B, also called Christmas disease, is an X-linked inherited bleeding disorder that predominantly affects males, and it is caused by deficiency of coagulation factor IX. Factor IX is a coagulation factor produced in the liver. Liver transplantation from hemophilia B donors is neither widely documented nor performed due to presumed risk of developing increased bleeding tendency in the recipient due to potential acquired factor IX deficiency. In this review, we present a case of liver and kidney transplantation from a donor with mild hemophilia B to a recipient with no history of hemophilia B and literature review. This is a relatively rare situation with only two other prior case reports of acquired Hemophilia B through liver transplantation noted in 2015. The 2 cases presented in the literature lead to acquired hemophilia B in liver transplant recipients since donors were not screened due to mild disease and low suspicion level. In our case, the donor was known to have mild Hemophilia B and the recipient had a rapid decline – thus it is imperative to weigh risks and benefits of transplantation from this donor population. While those individuals with mild hemophilia B disease (and low bleeding burden) should be considered as an alternative donor for liver transplantation, appropriate counseling should be done with the recipient about possible bleeding risks and OLT should only occur after patient consent. A multidisciplinary approach should involve hematology in case factor replacement therapy is needed post transplantation and for long term follow up.},
 year = {2020}
}

Copy | Download

TY  - JOUR
T1  - Acquired Hemophilia B Through Liver Transplantation: A Case Report and Literature Review
AU  - Christine Jane Kurian
AU  - Douglass Alan Drelich
AU  - Sanaa Rizk
Y1  - 2020/12/16
PY  - 2020
N1  - https://doi.org/10.11648/j.ajim.20200806.21
DO  - 10.11648/j.ajim.20200806.21
T2  - American Journal of Internal Medicine
JF  - American Journal of Internal Medicine
JO  - American Journal of Internal Medicine
SP  - 312
EP  - 315
PB  - Science Publishing Group
SN  - 2330-4324
UR  - https://doi.org/10.11648/j.ajim.20200806.21
AB  - Hemophilia B, also called Christmas disease, is an X-linked inherited bleeding disorder that predominantly affects males, and it is caused by deficiency of coagulation factor IX. Factor IX is a coagulation factor produced in the liver. Liver transplantation from hemophilia B donors is neither widely documented nor performed due to presumed risk of developing increased bleeding tendency in the recipient due to potential acquired factor IX deficiency. In this review, we present a case of liver and kidney transplantation from a donor with mild hemophilia B to a recipient with no history of hemophilia B and literature review. This is a relatively rare situation with only two other prior case reports of acquired Hemophilia B through liver transplantation noted in 2015. The 2 cases presented in the literature lead to acquired hemophilia B in liver transplant recipients since donors were not screened due to mild disease and low suspicion level. In our case, the donor was known to have mild Hemophilia B and the recipient had a rapid decline – thus it is imperative to weigh risks and benefits of transplantation from this donor population. While those individuals with mild hemophilia B disease (and low bleeding burden) should be considered as an alternative donor for liver transplantation, appropriate counseling should be done with the recipient about possible bleeding risks and OLT should only occur after patient consent. A multidisciplinary approach should involve hematology in case factor replacement therapy is needed post transplantation and for long term follow up.
VL  - 8
IS  - 6
ER  -

Copy | Download

Author Information

Christine Jane Kurian

Cardeza Foundation for Hematologic Research, Department of Medicine, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia PA, USA
Douglass Alan Drelich

Cardeza Foundation for Hematologic Research, Department of Medicine, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia PA, USA
Sanaa Rizk

Cardeza Foundation for Hematologic Research, Department of Medicine, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia PA, USA

Download PDF

Sections

Plain Text BibTeX RIS

APA Style

Christine Jane Kurian, Douglass Alan Drelich, Sanaa Rizk. (2020). Acquired Hemophilia B Through Liver Transplantation: A Case Report and Literature Review. American Journal of Internal Medicine, 8(6), 312-315. https://doi.org/10.11648/j.ajim.20200806.21

Copy | Download

ACS Style

Christine Jane Kurian; Douglass Alan Drelich; Sanaa Rizk. Acquired Hemophilia B Through Liver Transplantation: A Case Report and Literature Review. Am. J. Intern. Med. 2020, 8(6), 312-315. doi: 10.11648/j.ajim.20200806.21

Copy | Download

AMA Style

Christine Jane Kurian, Douglass Alan Drelich, Sanaa Rizk. Acquired Hemophilia B Through Liver Transplantation: A Case Report and Literature Review. Am J Intern Med. 2020;8(6):312-315. doi: 10.11648/j.ajim.20200806.21

Copy | Download

@article{10.11648/j.ajim.20200806.21,
  author = {Christine Jane Kurian and Douglass Alan Drelich and Sanaa Rizk},
  title = {Acquired Hemophilia B Through Liver Transplantation: A Case Report and Literature Review},
  journal = {American Journal of Internal Medicine},
  volume = {8},
  number = {6},
  pages = {312-315},
  doi = {10.11648/j.ajim.20200806.21},
  url = {https://doi.org/10.11648/j.ajim.20200806.21},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajim.20200806.21},
  abstract = {Hemophilia B, also called Christmas disease, is an X-linked inherited bleeding disorder that predominantly affects males, and it is caused by deficiency of coagulation factor IX. Factor IX is a coagulation factor produced in the liver. Liver transplantation from hemophilia B donors is neither widely documented nor performed due to presumed risk of developing increased bleeding tendency in the recipient due to potential acquired factor IX deficiency. In this review, we present a case of liver and kidney transplantation from a donor with mild hemophilia B to a recipient with no history of hemophilia B and literature review. This is a relatively rare situation with only two other prior case reports of acquired Hemophilia B through liver transplantation noted in 2015. The 2 cases presented in the literature lead to acquired hemophilia B in liver transplant recipients since donors were not screened due to mild disease and low suspicion level. In our case, the donor was known to have mild Hemophilia B and the recipient had a rapid decline – thus it is imperative to weigh risks and benefits of transplantation from this donor population. While those individuals with mild hemophilia B disease (and low bleeding burden) should be considered as an alternative donor for liver transplantation, appropriate counseling should be done with the recipient about possible bleeding risks and OLT should only occur after patient consent. A multidisciplinary approach should involve hematology in case factor replacement therapy is needed post transplantation and for long term follow up.},
 year = {2020}
}

Copy | Download

TY  - JOUR
T1  - Acquired Hemophilia B Through Liver Transplantation: A Case Report and Literature Review
AU  - Christine Jane Kurian
AU  - Douglass Alan Drelich
AU  - Sanaa Rizk
Y1  - 2020/12/16
PY  - 2020
N1  - https://doi.org/10.11648/j.ajim.20200806.21
DO  - 10.11648/j.ajim.20200806.21
T2  - American Journal of Internal Medicine
JF  - American Journal of Internal Medicine
JO  - American Journal of Internal Medicine
SP  - 312
EP  - 315
PB  - Science Publishing Group
SN  - 2330-4324
UR  - https://doi.org/10.11648/j.ajim.20200806.21
AB  - Hemophilia B, also called Christmas disease, is an X-linked inherited bleeding disorder that predominantly affects males, and it is caused by deficiency of coagulation factor IX. Factor IX is a coagulation factor produced in the liver. Liver transplantation from hemophilia B donors is neither widely documented nor performed due to presumed risk of developing increased bleeding tendency in the recipient due to potential acquired factor IX deficiency. In this review, we present a case of liver and kidney transplantation from a donor with mild hemophilia B to a recipient with no history of hemophilia B and literature review. This is a relatively rare situation with only two other prior case reports of acquired Hemophilia B through liver transplantation noted in 2015. The 2 cases presented in the literature lead to acquired hemophilia B in liver transplant recipients since donors were not screened due to mild disease and low suspicion level. In our case, the donor was known to have mild Hemophilia B and the recipient had a rapid decline – thus it is imperative to weigh risks and benefits of transplantation from this donor population. While those individuals with mild hemophilia B disease (and low bleeding burden) should be considered as an alternative donor for liver transplantation, appropriate counseling should be done with the recipient about possible bleeding risks and OLT should only occur after patient consent. A multidisciplinary approach should involve hematology in case factor replacement therapy is needed post transplantation and for long term follow up.
VL  - 8
IS  - 6
ER  -

Copy | Download