0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Special Feature |

Image of the Month—Diagnosis FREE

[+] Author Affiliations

Section Editor: Grace S. Rozycki, MD

More Author Information
Arch Surg. 2003;138(9):1026. doi:10.1001/archsurg.138.9.1025.
Text Size: A A A
Published online

ANSWER: OBTAIN A BIOPSY SPECIMEN OF THE HEPATIC MASS

Figure 1. Computed tomographic scan of the liver.

Figure 2. Magnetic resonance imaging of the liver.

The computed tomographic and magnetic resonance images demonstrated a 6.6-cm lesion of the medial and lateral segments in the left lobe of the liver, peripheral biliary ductal dilatation in both lobes, and obstruction of the common hepatic duct. A biopsy of this lesion was diagnostic of malignant B-cell lymphoma and was positive by in situ hybridization for the Epstein-Barr virus. Serologic analyses confirmed posttransplantation infection by the Epstein-Barr virus. Immunosuppression was discontinued, temporary biliary stenting via endoscopic retrograde cholangiopancreatography was accomplished, and chemotherapy with cyclophosphamide, adriamycin, rituximab, and prednisone was initiated. Five months later, the mass resolved, renal and hepatic function were preserved (serum creatinine, 1.6 mg/dL [141.4 µmol/L]); total bilirubin, 0.4 mg/dL [6.84 µmol/L]), and low-dose immunosuppression has been reinstituted with cyclosporine microemulsion and prednisone.

Posttransplantation lymphoproliferative disease is the second most common malignancy after solid organ transplantation (squamous cell carcinoma of the skin is the most common) and occurs in approximately 2% of recipients.1 The frequency of posttransplantation lymphoproliferative disease is increased by the high doses of immunosuppressive agents for induction or the treatment of rejection. The clinical signs and symptoms are highly variable and include lesions found in the allograft itself or, as in this case, in remote sites. The time of initial symptoms ranges from 1 month to many years posttransplantation, with a median onset of about 1 year.2 Demonstrably associated with the Epstein-Barr virus in 80% of cases, these lesions are usually of B-cell lineage and are believed to result from decreased immunosurveillance induced by pharmacologic immunosuppression.3 Treatment is based on the location and aggressiveness of the lymphoma itself and on whether the allograft can be sacrificed in stimulating recovery of the immune response. On occasion, reduction of the level of immunosuppression alone suffices to induce regression of the tumor(s).4 Other strategies that may be used in combination with reduced immunosuppression include chemotherapy, radiation, surgery (allograft removal may be appropriate), anti-viral medication (acyclovir, ganciclovir), alpha interferon, and newer therapies, including monoclonal antibodies targeting B-cell surface antigens and Epstein-Barr virus–specific cytotoxic lymphocytes. The prognosis of posttransplantation lymphoproliferative disease is difficult to predict due to the wide range of clinical scenarios involved, but reported survival rates range from 27% to 45%.5

Corresponding author and reprints: Amy L. Friedman, MD, Yale University School of Medicine, FMB 112, 333 Cedar St, New Haven, CT 06520 (e-mail: amy.friedman@yale.edu).

Penn  IHammond  WBrettschneider  LStarzl  TE Malignant lymphomas in transplantation patients. Transplant Proc. 1969;1106
PubMed
Nelson  BPNalesnik  MABahler  DW  et al.  Epstein-Barr virus-negative post-transplant lymphoproliferative disorders: a distinct entity? Am J Surg Pathol. 2000;24375- 385
PubMed Link to Article
Dotti  GFiocchi  RMotta  T  et al.  Lymphomas occuring late after solid-organ transplantation: influence of treatment on the clinical outcome. Transplantation. 2002;741095- 1102
PubMed Link to Article
Manz  MGBerger  CHorny  HP  et al.  Sustained remission of an extensive monoclonal, Epstein-Barr virus-associated diffuse large B cell lymphoma in a kidney-pancreas transplant recipient. Transplantation. 2002;73995- 997
PubMed Link to Article
Borges  EFerry  JAFriedmann  AM Epstein-Barr virus-negative precursor B cell lymphoblastic lymphoma after liver transplantation: a unique form of posttransplant lymphoproliferative disease. Transplantation. 2002;73541- 543
PubMed Link to Article

Tables

References

Penn  IHammond  WBrettschneider  LStarzl  TE Malignant lymphomas in transplantation patients. Transplant Proc. 1969;1106
PubMed
Nelson  BPNalesnik  MABahler  DW  et al.  Epstein-Barr virus-negative post-transplant lymphoproliferative disorders: a distinct entity? Am J Surg Pathol. 2000;24375- 385
PubMed Link to Article
Dotti  GFiocchi  RMotta  T  et al.  Lymphomas occuring late after solid-organ transplantation: influence of treatment on the clinical outcome. Transplantation. 2002;741095- 1102
PubMed Link to Article
Manz  MGBerger  CHorny  HP  et al.  Sustained remission of an extensive monoclonal, Epstein-Barr virus-associated diffuse large B cell lymphoma in a kidney-pancreas transplant recipient. Transplantation. 2002;73995- 997
PubMed Link to Article
Borges  EFerry  JAFriedmann  AM Epstein-Barr virus-negative precursor B cell lymphoblastic lymphoma after liver transplantation: a unique form of posttransplant lymphoproliferative disease. Transplantation. 2002;73541- 543
PubMed Link to Article

Correspondence

CME
Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections
PubMed Articles