DOI: 10.1097/TP.0b013e3181cea646

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PMID: 20440198

Issn Print: 0041-1337

Publication Date: 2010/05/15


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Acute Graft-Versus-Host Disease After Living Donor Liver Transplantation With Donor-Dominant One-Way Human Leukocyte Antigen Matching at Two Loci

Tetsunosuke Shimizu; Michihiro Hayashi; Yoshihiro Inoue; Koji Komeda; Mitsuhiro Asakuma; Fumitoshi Hirokawa; Mitsuhiko Iwamoto; Yoshiharu Miyamoto; Noboru Yonetani; Hiroh Saji; Hironori Haga; Nobuhiko Tanigawa

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Author Information: Department of General and Gastroenterological Surgery; Osaka Medical College; Osaka, Japan (Shimizu, Hayashi, Inoue, Komeda, Asakuma, Hirokawa, Iwamoto, Miyamoto, Tanigawa)

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Excerpt

A 50-year-old man received living donor liver transplantation (LDLT) for hepatitis C liver cirrhosis. The donor was his blood-type-identical, 23-year-old son. The human leukocyte antigen (HLA) serological class I and class II phenotype of the donor was one identical and homozygous in two loci (HLA-A [24,-], HLA-B [52,-], HLA-DR [15,-]), whereas the recipient's were HLA-A (24,-), HLA-B (52,59), HLA-DR (15,08). During the transplantation, 8 U of packed red blood cells and 8 U of fresh-frozen plasma were transfused. Immunosuppression consisted of tacrolimus, low-dose steroids, and mycophenolate mofetil.
On postoperative day (POD) 15, he developed a high fever and watery diarrhea (7–10 times, ∼1.5-2 L/day). Six days later (POD 21), he developed a skin rash on the trunk and feet. At the same time, neurological symptoms, such as restlessness, cognitive dysfunction, and abnormal behavior, with signs of meningeal irritation appeared. Initially, viral encephalomyelitis with dermatological symptoms was suspected based on the cerebrospinal fluid findings. Ganciclovir, acyclovir, immunoglobulin, and betamethasone were administered. Neurological and cerebrospinal fluid findings, as well as watery diarrhea and high fever, were improved on POD 26, but the skin rash did not change. However, on POD 30, neurological findings and high fever reappeared with deterioration of the skin rash, and pancytopenia and subcutaneous bleeding developed.
A bone marrow biopsy specimen revealed hemophagocytic syndrome on POD 35. A skin biopsy specimen suggested toxic epidermal necrosis or graft-versus-host disease (GVHD; Fig. 1) (POD 35). Meanwhile, the presence of donor-derived chimerism in the peripheral blood and skin was demonstrated by polymerase chain reaction of microsatellite marker, with peripheral blood chimerism being more than 95%. The diagnosis of GVHD was made on the basis of clinical symptoms, histologic findings, and the chimerism data. Then, HLA DNA typing revealed the following results: donor—HLA-A (2402,-), HLA-B (5201,-), HLA-DR (1502,-); recipient—HLA-A (2402,-), HLA-B (5201,5901), HLA-DR (1502,0803). We confirmed that the HLA class I and class II phenotype of the donor was one identical and homozygous in two loci. Treatment for GVHD was started with the combination of methylprednisolone and antithymocyte globulin, but the clinical status of the patient showed no improvement. The patient developed septic shock and multiple organ failure, and he died on POD 40.
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