Child, Preschool ; Cord Blood Stem Cell Transplantation ; adverse effects ; Humans ; Lymphoproliferative Disorders ; etiology ; Male

Child ; Epstein-Barr Virus Infections ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Herpesvirus 4, Human ; Humans ; Lymphoproliferative Disorders ; etiology ; virology ; Male

OBJECTIVETo evaluate clinical and pathological features of post-transplant lymphoproliferative disorders (PTLD) and its significance in diagnosis.

METHODSSix cases of PTLD were studied by light microscope, immunohistochemistry, in-situ hybridization, and gene rearrangement analysis. The clinical and follow-up information were also reviewed.

RESULTSAmong the 6 cases, 3 with monomorphic PTLD were renal transplant recipients, and died 4, 2, and 1 months after diagnosis. 2 were liver transplant recipients, 1 of whom with monomorphic PTLD died 5 months after diagnosis, the other one was diagnosed as early lesion of PTLD and the post-bone marrow transplant case was classified as polymorphic PTLD who survived for 12 months after diagnosis of PTLD. EBER 1/2 DNA was demonstrated in 4 cases.

CONCLUSIONSPTLD is a lymphoproliferative disease with distinctive morphologic and clinical characteristics after organ transplantation. The prognosis of PTLD correlates with the pathological subtypes and clinical stage.

Adult ; Female ; Follow-Up Studies ; Humans ; Lymphoproliferative Disorders ; etiology ; pathology ; Male ; Middle Aged ; Postoperative Complications ; pathology ; Prognosis ; Transplantation, Homologous ; pathology

A 16-yr-old girl received liver transplantation for fulminant hepatitis. Aplastic anemia developed, and she received hematopoietic stem cell transplantation (HSCT). Eleven months after liver transplantation, abdominal lymph node enlargement and colon ulcers were observed, and colon biopsy showed posttransplant lymphoproliferative disorder (PTLD). Immunosuppression reduction was attempted, but it produced no therapeutic effect. Fourteen months after liver transplantation, she received a second HSCT due to engraftment failure, and PTLD resolved completely. The second HSCT can serve as cellular therapy for PTLD.
Adolescent ; Female ; Hematopoietic Stem Cell Transplantation/*methods ; Humans ; Liver Transplantation/*adverse effects ; Lymphoproliferative Disorders/*etiology/*surgery ; Treatment Outcome

Epstein-Barr virus (EBV)-associated post-transplantation lymphoproliferative disease (EBV-PTLD) is a potentially life-threatening complication after hematopoietic stem cell transplantation or solid organ transplantation. In the last decade, the survival of patients with EBV-PTLD has been significantly improved by immunotherapeutic interventions among high-risk patients. The immunotherapeutic interventions for EBV-PTLD include reduction in immunosuppression, CD20 monoclonal antibodies (rituximab) as monotherapy or in combination with chemotherapy, and adoptive immunotherapy with EBV-specific T cells. This paper reviews the latest update on the high-risk factors, clinical manifestations and immunotherapy of EBV-PTLD.
Epstein-Barr Virus Infections ; complications ; therapy ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Immunotherapy ; methods ; Lymphoproliferative Disorders ; etiology ; therapy ; Postoperative Complications ; etiology ; therapy ; Risk Factors

Post-transplant lymphoproliferative disorder (PTLD) is one of the main complications after stem cell transplantation and is often induced by EBV. The optimal treatment of PTLD includes reduction of immunosuppressant dose, transplant organ resection, radiotherapy and chemotherapy, and so on. Recently, a new therapeutic approach was developed in PTLD: the anti-CD20 monoclonal antibody or rituximab. In this review, the application of rituximab in treatment of PTLD is summarized, including risk factors and mechanism of PTLD, therapeutic strategy, application of rituximab in PTLD and so on.
Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Lymphoproliferative Disorders ; drug therapy ; etiology ; Risk Factors ; Rituximab

B-Lymphocytes ; immunology ; Hepatitis B ; complications ; Humans ; Liver Transplantation ; adverse effects ; Lymphocyte Activation ; Lymphoproliferative Disorders ; etiology ; Male ; Middle Aged ; Risk Factors ; Virus Activation

Post-transplant lymphoproliferative disorders(PTLD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) are a group of rare, but are grievous complications. The occurrence of these diseases are most associated with EBV infection. The clinical manifestations usually include recurrent fever, lymph node enlargement, progressive decline of three lineage cells of hemogram, EB viremia and response failure to formal broad-spectrum antibiotics therapy, then the disease rapidly deteriorated in the short term, which result in high mortality. Therefore, early diagnosis and timely effective treatment such as rituximab, donor lymphocyte infusion and/or EB virus-specific cytotoxic T lymphocytes are needed to improve the prognosis. This review briefly summarized the diagnosis and therapy advance on the lymphoproliferative disorders after allogeneic hematopoietic stem cell transplantation.
Epstein-Barr Virus Infections ; diagnosis ; therapy ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Herpesvirus 4, Human ; Humans ; Lymphoproliferative Disorders ; diagnosis ; etiology ; therapy ; Transplantation, Homologous

Adolescent ; Adult ; Child ; Female ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Lymphoproliferative Disorders ; diagnosis ; etiology ; therapy ; Male ; Middle Aged ; Retrospective Studies ; Transplantation, Homologous ; Young Adult

Epstein-Barr virus (EBV) is a ubiquitous herpesvirus, affecting >90% of the adult population. EBV targets B-lymphocytes and achieves latent infection in a circular episomal form. Different latency patterns are recognized based on latent gene expression pattern. Latent membrane protein-1 (LMP-1) mimics CD40 and, when self-aggregated, provides a proliferation signal via activating the nuclear factor-kappa B, Janus kinase/signal transducer and activator of transcription, phosphoinositide 3-kinase/Akt (PI3K/Akt) and mitogen-activated protein kinase pathways to promote cellular proliferation. LMP-1 also induces BCL-2 to escape from apoptosis and gives a signal for cell cycle progression by enhancing cyclin-dependent kinase 2 and phosphorylation of retinoblastoma (Rb) protein and by inhibiting p16 and p27. LMP-2A blocks the surface immunoglobulin-mediated lytic cycle reactivation. It also activates the Ras/PI3K/Akt pathway and induces Bcl-xL expression to promote B-cell survival. Recent studies have shown that ebv-microRNAs can provide extra signals for cellular proliferation, cell cycle progression and anti-apoptosis. EBV is well known for association with various types of B-lymphocyte, T-lymphocyte, epithelial cell and mesenchymal cell neoplasms. B-cell lymphoproliferative disorders encompass a broad spectrum of diseases, from benign to malignant. Here we review our current understanding of EBV-induced lymphomagenesis and focus on biology, diagnosis and management of EBV-associated B-cell lymphoproliferative disorders.
B-Lymphocytes/*pathology/*virology ; Diagnosis, Differential ; Disease Management ; Epstein-Barr Virus Infections/*complications ; Herpesvirus 4, Human/*physiology ; Humans ; Lymphoproliferative Disorders/*diagnosis/*etiology/therapy