Suspected late graft failure and graft versus host disease 34 years after hematopoietic stem cell transplantation clinically and pathologically presenting as host versus graft disease with liver injury
A 36-year-old man who underwent hematopoietic stem cell transplantation (HSCT) at the age of 2 years for severe combined immunodeficiency, presented with jaundice, skin rash, and elevated liver function tests 34 years after HSCT. Liver biopsy showed bile duct injury and cholestasis. Viral studies, a...
Saved in:
| Main Authors: | , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2025-06-01
|
| Series: | Human Pathology Reports |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2772736X25000064 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849434437420318720 |
|---|---|
| author | Ejas Palathingal Bava Sharon Skorupski Edward Peres Pegah Dejban Qing Chang Brian Theisen Sanam Husain |
| author_facet | Ejas Palathingal Bava Sharon Skorupski Edward Peres Pegah Dejban Qing Chang Brian Theisen Sanam Husain |
| author_sort | Ejas Palathingal Bava |
| collection | DOAJ |
| description | A 36-year-old man who underwent hematopoietic stem cell transplantation (HSCT) at the age of 2 years for severe combined immunodeficiency, presented with jaundice, skin rash, and elevated liver function tests 34 years after HSCT. Liver biopsy showed bile duct injury and cholestasis. Viral studies, autoimmune panel, review of medications, and imaging did not establish a cause of liver injury. However, graft-versus-host disease (GVHD) was unlikely because of the remote history of HSCT. Short tandem repeat-polymerase chain reaction (STR-PCR) chimerism analysis showed that the percentage of donor DNA in the liver biopsy specimen was very low (11 %); hence, host-versus-graft disease (HVGD) was implicated. Because STR analysis of patient’s blood showed a mixed chimera with 11 % donor DNA, graft failure was suspected; however, fractionated STR analysis ruled out complete graft failure. Overall, this case outlines liver injury caused by HVGD in the absence of complete graft failure 34 years after HSCT, which has never been reported in the literature. STR-PCR analysis was essential for mitigating the diagnostic dilemma. |
| format | Article |
| id | doaj-art-bb7df96de5bd4855b4c4aeed18b46213 |
| institution | Kabale University |
| issn | 2772-736X |
| language | English |
| publishDate | 2025-06-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Human Pathology Reports |
| spelling | doaj-art-bb7df96de5bd4855b4c4aeed18b462132025-08-20T03:26:39ZengElsevierHuman Pathology Reports2772-736X2025-06-014030077410.1016/j.hpr.2025.300774Suspected late graft failure and graft versus host disease 34 years after hematopoietic stem cell transplantation clinically and pathologically presenting as host versus graft disease with liver injuryEjas Palathingal Bava0Sharon Skorupski1Edward Peres2Pegah Dejban3Qing Chang4Brian Theisen5Sanam Husain6Department of Pathology and Laboratory Medicine, Henry Ford Hospital, Detroit, MI, USADepartment of Pathology and Laboratory Medicine, Henry Ford Hospital, Detroit, MI, USADepartment of Hematology and Oncology, Henry Ford Hospital, Detroit, MI, USADepartment of Pathology and Laboratory Medicine, Henry Ford Hospital, Detroit, MI, USADepartment of Pathology and Laboratory Medicine, Henry Ford Hospital, Detroit, MI, USADepartment of Pathology and Laboratory Medicine, Henry Ford Hospital, Detroit, MI, USADepartment of Pathology and Laboratory Medicine, Henry Ford Hospital, Detroit, MI, USA; Corresponding author at: Department of Pathology, Henry Ford Hospital, Senior Staff Pathologist, Henry Ford Health System, Clinical Associate Professor, Department of Medicine, Michigan State University, 2799 W. Grand Blvd., Pathology - K-6, Detroit, MI 48202 USA.A 36-year-old man who underwent hematopoietic stem cell transplantation (HSCT) at the age of 2 years for severe combined immunodeficiency, presented with jaundice, skin rash, and elevated liver function tests 34 years after HSCT. Liver biopsy showed bile duct injury and cholestasis. Viral studies, autoimmune panel, review of medications, and imaging did not establish a cause of liver injury. However, graft-versus-host disease (GVHD) was unlikely because of the remote history of HSCT. Short tandem repeat-polymerase chain reaction (STR-PCR) chimerism analysis showed that the percentage of donor DNA in the liver biopsy specimen was very low (11 %); hence, host-versus-graft disease (HVGD) was implicated. Because STR analysis of patient’s blood showed a mixed chimera with 11 % donor DNA, graft failure was suspected; however, fractionated STR analysis ruled out complete graft failure. Overall, this case outlines liver injury caused by HVGD in the absence of complete graft failure 34 years after HSCT, which has never been reported in the literature. STR-PCR analysis was essential for mitigating the diagnostic dilemma.http://www.sciencedirect.com/science/article/pii/S2772736X25000064Graft-versus-host disease (GVHD)Hematopoietic stem cell transplantation (HSCT)Short tandem repeat-polymerase chain reaction (STR-PCR) chimerism analysisHost-versus-graft disease (HVGD)Liver injury |
| spellingShingle | Ejas Palathingal Bava Sharon Skorupski Edward Peres Pegah Dejban Qing Chang Brian Theisen Sanam Husain Suspected late graft failure and graft versus host disease 34 years after hematopoietic stem cell transplantation clinically and pathologically presenting as host versus graft disease with liver injury Human Pathology Reports Graft-versus-host disease (GVHD) Hematopoietic stem cell transplantation (HSCT) Short tandem repeat-polymerase chain reaction (STR-PCR) chimerism analysis Host-versus-graft disease (HVGD) Liver injury |
| title | Suspected late graft failure and graft versus host disease 34 years after hematopoietic stem cell transplantation clinically and pathologically presenting as host versus graft disease with liver injury |
| title_full | Suspected late graft failure and graft versus host disease 34 years after hematopoietic stem cell transplantation clinically and pathologically presenting as host versus graft disease with liver injury |
| title_fullStr | Suspected late graft failure and graft versus host disease 34 years after hematopoietic stem cell transplantation clinically and pathologically presenting as host versus graft disease with liver injury |
| title_full_unstemmed | Suspected late graft failure and graft versus host disease 34 years after hematopoietic stem cell transplantation clinically and pathologically presenting as host versus graft disease with liver injury |
| title_short | Suspected late graft failure and graft versus host disease 34 years after hematopoietic stem cell transplantation clinically and pathologically presenting as host versus graft disease with liver injury |
| title_sort | suspected late graft failure and graft versus host disease 34 years after hematopoietic stem cell transplantation clinically and pathologically presenting as host versus graft disease with liver injury |
| topic | Graft-versus-host disease (GVHD) Hematopoietic stem cell transplantation (HSCT) Short tandem repeat-polymerase chain reaction (STR-PCR) chimerism analysis Host-versus-graft disease (HVGD) Liver injury |
| url | http://www.sciencedirect.com/science/article/pii/S2772736X25000064 |
| work_keys_str_mv | AT ejaspalathingalbava suspectedlategraftfailureandgraftversushostdisease34yearsafterhematopoieticstemcelltransplantationclinicallyandpathologicallypresentingashostversusgraftdiseasewithliverinjury AT sharonskorupski suspectedlategraftfailureandgraftversushostdisease34yearsafterhematopoieticstemcelltransplantationclinicallyandpathologicallypresentingashostversusgraftdiseasewithliverinjury AT edwardperes suspectedlategraftfailureandgraftversushostdisease34yearsafterhematopoieticstemcelltransplantationclinicallyandpathologicallypresentingashostversusgraftdiseasewithliverinjury AT pegahdejban suspectedlategraftfailureandgraftversushostdisease34yearsafterhematopoieticstemcelltransplantationclinicallyandpathologicallypresentingashostversusgraftdiseasewithliverinjury AT qingchang suspectedlategraftfailureandgraftversushostdisease34yearsafterhematopoieticstemcelltransplantationclinicallyandpathologicallypresentingashostversusgraftdiseasewithliverinjury AT briantheisen suspectedlategraftfailureandgraftversushostdisease34yearsafterhematopoieticstemcelltransplantationclinicallyandpathologicallypresentingashostversusgraftdiseasewithliverinjury AT sanamhusain suspectedlategraftfailureandgraftversushostdisease34yearsafterhematopoieticstemcelltransplantationclinicallyandpathologicallypresentingashostversusgraftdiseasewithliverinjury |