Posttransplant Lymphoproliferative Disorder in Children: A 360-degree Perspective

Abstract

Posttransplant lymphoproliferative disorder (PTLD) is the most common malignancy that can occur after organ transplant in children. PTLD arises because the patient's immune system has been suppressed to protect the graft and may fail to provide an adequate immune check for transformed malignant or premalignant lymphocytes. PTLD risk factors and pathogenesis are not completely understood; however, Epstein-Barr virus is identified in many patients with PTLD and may contribute to its evolution. PTLD is a clinical challenge because the biologic behavior and clinical manifestations are diverse, and there is no standardized treatment. Treatment options include reduction of immunosuppression therapy, chemotherapy, radiation therapy, and surgical resection of localized lesions, depending on factors including the type and extent of disease, whether the patient has positive test results for the Epstein-Barr virus, and patient comorbidities. A multidisciplinary approach is essential for the appropriate management of PTLD. Diagnostic imaging modalities such as radiography, US, CT, MRI, and PET are essential in diagnosis, assessment of therapeutic response, and monitoring of this heterogeneous disease entity. When imaging findings are suggestive of PTLD, prompt tissue biopsy to identify the PTLD subtype is essential for appropriate treatment. It is important to know the proper indications and limitations of different diagnostic imaging techniques in the management of PTLD. In addition, familiarity with the imaging features of PTLD and its mimics narrows the differential diagnosis and may facilitate decision making about patient treatment. ©

Epstein–Barr Viral Load Monitoring Strategy and the Risk for Posttransplant Lymphoproliferative Disease in Adult Liver Transplantation

“…This difference between adults and children is mainly related to the higher incidence of primary EBV infection after LT in children (1-3, 6, 8-11). In children and adolescents, the risk factors for PTLD are EBV seronegativity, immunosuppression intensity, and first year after transplant (6,(8)(9)(10)(11). In adult recipients, PTLD can occur later after LT, and risk factors are less well known (1)(2)(3)10).…”

Die Rolle der [18F]FDG PET/CT bei pädiatrischen Lymphomen

“…bei Kindern, insbesondere im ersten Jahr nach erfolgter Transplantation[23]. PTLD werden bei etwa 25 % der transplantierten Kinder beobachtet.…”

Gastric Post-Transplant Lymphoproliferative Disorder after Allogenic Stem Cell Transplantation

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Post-Transplant Lymphoproliferative Disease (PTLD) is a well-known complication following transplantation. Its estimated incidence is 2.5% at 1 year after bone marrow transplantation [1,2]. PTLD consists of excessive (most frequently B) lymphocyte proliferation in immunosuppressed patients.

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“…Staging ranges from plasmocytic hyperplasia to lymphoma. PTLD can be potentially fatal and treatment is based on reduced immunosuppression, antiviral therapies, B cell proliferation blockers, chemotherapy and most recently T-cell therapies [1,3]. We present the case of an 18-year-old young man, who presented with a 48-hour history of low-grade fever, anorexia, abdominal discomfort and vomiting.…”

“…Unfortunately the PTLD was progressive, refractory to different therapies, and fatal in 5 months after PTLD diagnosis. Gastric PTLD is a very rare entity with only a few cases having been reported in the literature [1,4]. Diagnosis and management of EBV gastrointestinal PTLD is difficult in many patients, but a multidisciplinary approach and a prompt biopsy to identify the PTLD subtype are important for appropriate therapy.…”