Late Onset of Severe Graft-Versus-Host Disease in a Pediatric Liver Transplant Recipient

Abstract

We report the management of a patient with the late onset of chronic graft-versus-host disease (GVHD) after orthotopic liver transplantation. GVHD is a rare complication of solid organ transplants that usually presents early after transplantation and is fatal in the majority of cases. Our patient differs from the typical patient with GVHD in that the onset of her disease was very late. Although most treatment to date consisted of an increase in immunosuppressive therapy, our patient showed an excellent response to a reduction. This resulted in the abatement of the symptoms of GVHD and the preservation of her allograft function.

Graft versus Host Disease after Orthotopic Liver Transplantation Documented by Analysis of Short Tandem Repeat Polymorphisms

“…In the early postoperative period macrochimerism is transient and disappears 3–4 weeks after transplantation. Only macrochimerism detected later is associated with GVHD [6]. In our patient a bone marrow sample taken on day 72 after OLT was available for STR analysis done in retrospect.…”

“…Our treatment efforts were high-dose corticosteroids in the beginning after diagnosis; later on, as no therapeutic response was seen, immunosuppressive therapy was reduced and corticosteroids were withdrawn [4,5,6]. However, it can only be speculated whether a complete secession of immunosuppressive treatment, which was not done in our case, would have been able to mount a sufficiently strong immune response towards minor histocompatibility antigens of the donor lymphocytes to eliminate them successfully.…”

“…We used high-dose corticosteroids in the beginning after the diagnosis of GVHD; later on, as no therapeutic response was seen, immunosuppressive therapy was reduced and corticosteroids were withdrawn [4,5,6]. Thrombocytopenia had to be corrected frequently with thrombocyte concentrates and neutropenia responded in the beginning favorably to the administration of colony-stimulating factor G-CSF (filgrastim) with an increase in neutrophil counts as seen by others.…”

Severe, late-onset graft-versus-host disease in a liver transplant recipient documented by chimerism analysis

“…For these patients, only about 3% donor cells were detected in the PB, and both patients responded to temporary discontinuation of immunosuppressive therapy. Dunn et al [3] described another child who presented 5 years after liver transplantation with intestinal and skin symptoms consistent with GVHD. Her PB revealed (only) 1% male cells, but because she had received six of 16 units of unirradiated blood from male donors, the diagnosis of GVHD could not be attributed to liver donor lymphocytes; the patient quickly recovered after treatment with a small pulse of steroids.…”

“…A review of the literature indicated that both increasing and decreasing immunosuppression [1][2][3] have been used successfully in treating organ-transplant-related acute GVHD, although outcomes have generally been poor, regardless of treatment, with a mortality that exceeded 75% [1]. In this case, a course of OKT3 was provided as therapy for the GVHD.…”