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      Post-transplantation lymphoproliferative disorder with gastrointestinal involvement

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          Abstract

          Post-transplantation lymphoproliferative disorders (PTLDs) are lymphoid proliferations or lymphomas that are the second most common tumors in adult transplant recipients. Most cases of PTLD are attributed to Epstein-Barr virus, which induces B-cell proliferation and occurs in the setting of severe immunosuppression after solid organ or bone marrow transplantation. The disorder is seen in 1-3% of liver transplant recipients and has a variable presentation chronology. Herein, we chronicle a case of aggressive B-cell lymphoma (PTLD WHO class-3) presenting with isolated gastrointestinal involvement in an Epstein-Barr virus-negative patient with living-donor liver transplantation, 4 years after receiving the transplant. While typical symptoms may be elusive in the immunocompromised setting, clinicians should be vigilant for underlying PTLD with isolated gastrointestinal involvement. Prompt detection and characterization by endoscopic evaluation with biopsy should be particularly stressed in such patients.

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          Post-transplant lymphoproliferative disease (PTLD): risk factors, diagnosis, and current treatment strategies.

          Zeina Al-Mansour, Beverly Nelson, Andrew M Evens (2013)
          Post-transplant lymphoproliferative diseases (PTLD) are heterogeneous lymphoid disorders ranging from indolent polyclonal proliferations to aggressive lymphomas that complicate solid organ or hematopoietic transplantation. Risk factors for PTLD include viral infections, degree of immunosuppression, recipient age and race, allograft type, and host genetic variations. Clinically, extra-nodal disease is common including 10-15 % presenting with central nervous system (CNS) disease. Most PTLD cases are B cell (5-10 % T/NK cell or Hodgkin lymphoma), while over one-third are EBV-negative. World Health Organization (WHO) diagnostic categories are: early lesions, polymorphic, and monomorphic PTLD; although in practice, a clear separation is not always possible. Therapeutically, reduction in immunosuppression remains a mainstay, and recent data has documented the importance of rituximab +/- combination chemotherapy. Therapy for primary CNS PTLD should be managed according to immunocompetent CNS paradigms. Finally, novel treatment strategies for PTLD have emerged, including adoptive immunotherapy and rational targeted therapeutics (e.g., anti-CD30 based therapy and downstream signaling pathways of latent membrane protein-2A).
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            Risk factors for early-onset and late-onset post-transplant lymphoproliferative disorder in kidney recipients in the United States.

            Scott Quinlan, Ruth Pfeiffer, Josephine Engels … (2011)
            Solid-organ transplant recipients have an elevated risk for some malignancies because of the requirement for immunosuppression [1]. In particular, non-Hodgkin's lymphoma (NHL) is common and comprises one end of a spectrum of post-transplant lymphoproliferative disorder (PTLD) ranging from benign hyperplasia to lymphoid malignancy [2]. PTLD risk is influenced by the type of organ transplanted, the age and Epstein-Barr virus (EBV) serostatus of the transplant recipient, and the intensity of immunosuppression [3-9]. PTLD incidence is high immediately after transplantation, decreases subsequently, and then rises again 4-5 years from transplantation [10,11]. This incidence pattern suggests the presence of separate early-onset and late-onset PTLD subtypes. Early-onset PTLDs tend to be EBV-positive and, when extranodal, are more likely than late-onset PTLDs to be localized to the transplanted organ [12,13]. Late-onset PTLD is less likely to be associated with EBV and, overall, is more likely than early-onset PTLD to be extranodal [13,14]. The Scientific Registry of Transplant Recipients (SRTR) includes data on a large number of solid-organ transplant recipients in the United States and information on malignancies diagnosed post-transplantation. We used these data to conduct a retrospective cohort study among kidney transplant recipients to examine differences in risk factors between early-onset PTLD and late-onset PTLD.
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              Lymphoma after solid organ transplantation: risk, response to therapy, and survival at a transplantation center.

              D M Cibrik, Christopher J. Knight, Alexander Tsodikov … (2009)
              We studied the incidence, risk factors, treatment, and outcomes of post-transplantation lymphoproliferative disorder (PTLD) that occurred at the University of Michigan since 1964. We identified 7,040 patients who received solid organ transplantation (SOT) and post-transplantation immunosuppressive therapy. Seventy-eight patients developed PTLD. Diffuse large B-cell lymphoma (n = 43), polymorphic PTLD (n = 10), Hodgkin's lymphoma (n = 7), Burkitts lymphoma (n = 6), plasmacytoma (n = 5), and mucosa-associated lymphoid tissue lymphoma (n = 3) were all over-represented in the SOT population compared with a population sample from the Surveillance, Epidemiology, and End Results (SEER) database; follicular lymphoma (n = 0) was underrepresented. Negative pretransplantation Epstein-Barr virus (EBV) serology was a risk factor for PTLD. Available histologic analysis of tumor tissue showed that 75% were CD20 positive and that 62% were EBV positive; EBV-positive tumors occurred sooner after SOT than EBV-negative tumors (mean, 29 v 66 months). Extralymphatic disease (79%), poor performance status (68%), elevated lactate dehydrogenase (LDH; 71%), and advanced stage (68%) disease were all common at the time of lymphoma diagnosis. Two thirds of patients had a complete response when treated with cyclophosphamide, doxorubicin, vincristine, and prednisone-like chemotherapy (either with or without rituximab). Median overall survival in all patients with PTLD was 8.23 years (95% CI, 2.28 to 30.0 years). EBV-naïve patients who receive a donor organ from an EBV-infected donor are in the highest-risk situation for PTLD development. Most of these lymphomas are CD20 positive. Follicular lymphoma is unusual. With treatment, survival of patients with PTLD was indistinguishable from that of the SEER population sample.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Ann Gastroenterol
                Journal ID (iso-abbrev): Ann Gastroenterol
                Title: Annals of Gastroenterology
                Publisher: Hellenic Society of Gastroenterology (Greece )
                ISSN (Print): 1108-7471
                ISSN (Electronic): 1792-7463
                Publication date (Print): Mar-Apr 2018
                Publication date (Electronic): 08 January 2018
                Volume: 31
                Issue: 2
                Pages: 248-251
                Affiliations
                [a ]Allama Iqbal Medical College, Lahore, Pakistan (Faisal Inayat)
                [b ]Ameer ud Din Medical College, Lahore, Pakistan (Ghias UI Hassan, Ghias Un Nabi Tayyab)
                [c ]Tufts University School of Medicine, Boston, MA, USA (Muhammad Wasif Saif)
                Author notes
                Correspondence to: Muhammad Wasif Saif, MD, Director, GI Oncology Program; Leader, Experimental Therapeutics, Tufts Medical Center, South Building, 8 th Floor; 800 Washington St., Box 245, Boston, MA 02111, USA, email: wsaif@ 123456tuftsmedicalcenter.org
                Article
                Publisher ID: AnnGastroenterol-31-248
                DOI: 10.20524/aog.2018.0226
                PMC ID: 5825960
                PubMed ID: 29507477
                SO-VID: 5fc63580-d61e-49d8-96e6-3bb808c613c6
                Copyright © Copyright: © Hellenic Society of Gastroenterology
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 09 October 2017
                Date accepted : 05 November 2017
                Categories
                Subject: Image of the Month

                Keywords: post-transplantation lymphoproliferative disorder,gastrointestinal,intussusception,iron-deficiency anemia,awareness,treatment
                Data availability:
                Keywords: post-transplantation lymphoproliferative disorder, gastrointestinal, intussusception, iron-deficiency anemia, awareness, treatment

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