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At least 19 recordsLinked to original sources
PubMed2006

[Inheritance in lymphoproliferative disorders].

Lymphoproliferative disorders, especially chronic lymphocytic leukaemia (CLL), non-Hodgkin's lymphomas, Hodgkin's lymphoma and multiple myeloma are regarded as a hereditary entity with pleiotropic clustering in families, although the genuine alleles have not been found so far. The world-wide highest incidence of CLL and the existence of a systematic cancer registration since 1943 make Denmark a perfect place for epidemiological and genealogical investigations in the search for the genetics of the lymphoproliferative disorders. In Scandinavia, we see no signs of anticipation but marked linkage between parents and children, where the combination CLL in parent and CLL in child is more predominant than CLL in parent and a child with any other type of lymphoproliferative disorder. This same conservative pattern is also seen in parent-children transportation of non-Hodgkin's lymphomas and Hodgkin's lymphoma. That no certain linkage to other cancers can be significantly detected is discussed. A non-Mendelian mode of inheritance seems not unlikely in the familial clustering of the lymphoproliferative disorders.

Denmark↗
PubMed2005

Vaccine strategies to treat lymphoproliferative disorders.

Lymphoproliferative disorders, including follicular lymphoma (FL), multiple myeloma (MM) and chronic lymphatic leukaemia (CLL), are slowly progressive malignancies which remain incurable despite advances in therapy. Harnessing the immune system to recognise and destroy tumours is a promising new approach to treating these diseases. Dendritic cells (DC) are unique antigen-presenting cells that play a central role in the initiation and direction of immune responses. DC loaded ex vivo with tumour-associated antigens and administered as a vaccine have already shown promise in early clinical trials for a number of lymphoproliferative disorders, but the need for improvement is widely agreed. Recent advances in the understanding of basic DC biology and lessons from early clinical trials have provided exciting new insights into the generation of anti-tumour immune responses and the design of vaccine strategies. In this review we provide an overview of our current understanding of DC biology and their function in patients with lymphoproliferative disorders. We discuss the current status of clinical trials and new approaches to exploit the antigen presenting capacity of DC to design vaccines of the future.

Cancer Vaccines↗
PubMed1993

Molecular genetic analysis of post-transplant lymphoproliferative disorders.

Lymphoproliferative disorders developing in the post-transplant setting are a devastating set of diseases with major implications for the patients who develop them. On the other hand, they have provided us with an opportunity to better understand the basic biology of these disorders and malignant lymphomas in general. Molecular gene rearrangement studies and the Epstein-Barr virus, which is found in the genome of most of the abnormal lymphocytes in these disorders, have been investigated to further our understanding of the development and progression of these diseases.

Gene Rearrangement↗
PubMed2006

Immune-cell treatment of Epstein--Barr-virus-associated lymphoproliferative disorders.

Lymphoproliferative disorders associated with Epstein--Barr virus (EBV) after bone-marrow or organ transplantation express all the immunogenic EBV antigens, and reduction in immunosuppressive treatment can result in permanent resolution. As such, the disease lends itself to EBV-directed immune-cell therapy. Successes have been achieved with both manipulated and unmanipulated T-cell infusions for lymphoproliferations occurring after bone-marrow transplantation. Several practical challenges have been overcome in applying EBV-specific T-cell therapy to the setting of organ-transplant-related lymphoproliferations. These include the generation of autologous cytotoxic T lymphocytes (CTLs), the creation of a partially HLA-matched cryopreserved allogeneic CTL bank, and the generation of autologous EBV-specific CTLs from EBV-naïve pediatric patients. The efficacy of immune-cell therapy in the setting of solid-organ transplantation is less well established than it is after T-cell-depleted allogeneic bone-marrow transplantation, and it is as yet not clear how to best to integrate CTL therapy with the anti-B-cell antibody rituximab, which has significant activity against these lymphoproliferations.

Bone Marrow Transplantation↗
PubMed1997

Histologic, molecular, and radiologic characterization of resolving cerebral posttransplant lymphoproliferative disorder.

Lymphoproliferative disorders (LPDs) are commoner in pediatric versus adult immunosuppressed transplant recipients, and frequently involve the central nervous system. In these circumstances, the justification for biopsy is heavily influenced by the likely diagnostic yield. The present study centers on a 12-y-old renal transplant patient who developed multifocal cerebral LPD and had serial magnetic resonance (MR) examinations during the course of her illness from which she has completely recovered upon reduction of immunosuppression. She underwent stereotaxic biopsy, which was analyzed by both immunocytochemistry and polymerase chain reaction to examine the general question of how to release the maximum amount of information contained within, as well as to obtain a tissue diagnosis in this particular case. We show that a combination of these methods permits identification of the immunophenotype, lineage, clonality, viral involvement, and origin of abnormal cellular infiltrates. The biopsy also showed a novel histologic pattern of LPD, comprising numerous benign T cells obscuring a tiny clone of B cells. The MR examinations documented, for the first time, the differences in signal that accompany clinical resolution at both biopsied and nonbiopsied sites, showing that the latter may be associated with reduction, but not elimination, of MR signal abnormality. We conclude: 1) a combination of conventional and polymerase chain reaction analysis offers the greatest diagnostic yield from stereotaxic biopsies, even when the available tissue is minimal; 2) a focal polyclonal T cell infiltrate should prompt further investigation to exclude an underlying B cell lesion.

Adult↗
PubMed1995

B-cell lymphoproliferative disorder complicated by a natural killer cell lymphoproliferative disorder of granular lymphocytes associated with T-cell gene rearrangement: case report and review of the literature.

We report an unusual case of natural killer cell lymphoproliferative disorder of granular lymphocytes associated with a T-cell receptor (TCR)-beta gene rearrangement. The patient developed the disorder 1 month after cessation of fludarabine therapy for a B-cell lymphoproliferative disorder. The B-cell lymphoproliferative disorder was no longer detectable when the natural killer cell lymphoproliferative disorder persisted. Review of the literature reveals only one reported case of natural killer cell lymphoproliferative disorder of granular lymphocytes associated with a TCR-delta gene rearrangement.

B-Lymphocytes↗
PubMed2003

Posttransplantation lymphoproliferative disorders.

Posttransplantation lymphoproliferative disorders include a wide spectrum of diseases ranging from hyperplastic-appearing lesions to frank non-Hodgkin lymphoma. More than 90% of these disorders are Epstein-Barr virus-associated lesions of B-cell origin that arise in the setting of pharmacologic immunosuppression after transplantation. With the increased use of organ transplantation and intensive immunosuppression, posttransplantation lymphoproliferative disorders are becoming more common. The prognosis is often poor, with most patients dying despite receiving treatment. The aim of this review is to report the most recent knowledge about the clinical features, diagnosis, prophylaxis, and treatment of posttransplantation lymphoproliferative disorders, which can be useful to physicians and health assistants dealing with these life-threatening, posttransplantation clinical entities in clinical practice.

Epstein-Barr Virus Infections↗
PubMed1997

Chronic lymphoproliferative disorders.

Chronic lymphoproliferative disorders are a heterogeneous group of diseases with a highly variable clinical course. In the past few years, important progress has been made in the classification, biology, and therapy of some of these disorders of which B-cell chronic lymphocytic leukemia is not only the most frequent but also the model for their study. Regarding classification, entities that may be confounded with chronic lymphocytic leukemia have been identified (eg, splenic lymphoma with villous lymphocytes, mantle-cell lymphoma in leukemic phase); these diseases should be known and clearly separated from chronic lymphocytic leukemia because their prognosis and treatment is different from that of chronic lymphocytic leukemia. On the other hand, the molecular basis of some of these diseases (eg, the overexpression of the Prad1/CCND1 gene in mantle-cell lymphomas, the relationship between bcl-2 and bax expression in chronic lymphocytic leukemia homeostasis, the role of p53 tumor suppressor gene mutations in chronic lymphocytic leukemia progression) are increasingly well known. Cytokines (eg, tumor necrosis factor-alpha or IL-2, IL-4, and IL-13) also contribute to the pathogenesis of lymphoproliferative disorders by either promoting cell growth or inhibiting apoptosis. In addition, new treatment possibilities (eg, purine analogues, hemopoietic progenitors transplants) are changing the treatment objectives in some of these diseases. Thus, symptoms' palliation is no longer the only realistic aim in the management of patients with chronic lymphoproliferative disorders, but sustained complete remissions and even cures can be achieved.

Antineoplastic Agents↗
PubMed1993

Pulmonary lymphoproliferative disorders.

Pulmonary lymphoproliferative disorders are a diverse group of conditions that primarily or secondarily involve the lungs. Modern diagnostic modalities, including immunologic and molecular genetics techniques, have contributed substantially to the continuously evolving understanding of these complex lesions. In this review, we classified pulmonary lymphoproliferative disorders into benign, intermediate or indeterminate, and malignant categories and described the clinical, radiographic, and pathologic features for the entire spectrum. Our emphasis is on the clinical findings and the role of tissue biopsy as well as ancillary techniques in diagnosis. Various treatments and expected responses are also discussed.

Humans↗
PubMed1990

Involvement of the gastrointestinal tract by Epstein-Barr virus--associated posttransplant lymphoproliferative disorders.

Posttransplant lymphoproliferative disorders (PTLD) are abnormal growths of lymphoid cells that occur in immunosuppressed organ transplant recipients. Most cases are of B-lymphocyte origin and are associated with Epstein-Barr virus infection. Twelve of 72 allograft recipients with PTLD in our series have had disease predominantly involving the gastrointestinal tract. The lesions are most often multiple and preferentially involve the distal small bowel. The appearance of the lymphoid cells ranges from nonuniform (polymorphic) to uniform (monomorphic). Most tumors contain a clonal component of B-lymphocytes, with or without a nonclonal background. Appropriate primary treatment includes surgical resection and reduction of immunosuppression. Occasional PTLD of the gastrointestinal tract do not respond to this regimen, these represent a more advanced state of tumor progression. Currently, 11 of 12 patients are alive 10-13 months after diagnosis. The surgical pathologist must be aware of the appropriate setting in which to consider a diagnosis of PTLD and be able to distinguish this condition from other lymphoproliferative disorders of the gastrointestinal tract.

Adolescent↗
PubMed1997

Molecular pathology of posttransplantation lymphoproliferative disorders.

Posttransplantation lymphoproliferative disorders (PT-LPDs) represent a heterogeneous group of Epstein-Barr virus (EBV) associated lymphoid proliferations occurring in the setting of immunosuppression associated with solid organ transplantation. Some PT-LPDs regress after a reduction in immunosuppression, whereas others progress despite aggressive therapy. Previously defined histopathologic categories do not correlate with clonality, and neither histopathology nor clonality has reliably predicted their clinical behavior. Recently, correlative clinical, morphological, and molecular genetic analysis has suggested that PT-LPDs are divisible into three distinct clinically relevant categories as follows: (1) plasmacytic hyperplasia: most commonly arise in the oropharynx or lymph nodes, are nearly always polyclonal, usually contain multiple EBV infectious events or only a minor cell population infected by a single form of EBV, and lack oncogene or tumor suppressor gene alterations; (2) polymorphic lymphoproliferative disorders: may arise in lymph nodes or extranodal sites including the gastrointestinal tract and lungs, are nearly always monoclonal based on the presence of clonal immunoglobulin gene rearrangements, usually contain a single form of EBV, and lack oncogene or tumor suppressor gene alterations; and (3) malignant lymphoma or multiple myeloma: present with widely disseminated disease frequently including the bone marrow, are monoclonal based on clonal immunoglobulin gene rearrangements, contain a single form of EBV, and contain alterations of one or more oncogenes or tumor suppressor genes (c-myc, ras, p53). Thus, proto-oncogene and tumor suppressor gene alterations appear to be associated with disease progression and an often fatal clinical outcome. Furthermore, multiple PT-LPD lesions occurring in the same individual but in multiple anatomic sites, either simultaneously or dysynchronously over time, may show distinct clonal immunoglobulin gene rearrangement patterns and evidence of infection by different forms of EBV, suggesting that each lesion represents a distinct clonal neoplasm that may show distinctive clinical behavior. Therefore, whenever possible, a biopsy of each one of the several PT-LPD lesions occurring in an individual should be obtained to derive a true assessment of the pathobiological nature and clinical aggressiveness of an individual's disease.

Herpesviridae Infections↗
PubMed2003

Long-term follow-up of kidney transplant patients with posttransplant lymphoproliferative disorder: duration of posttransplant lymphoproliferative disorder-induced operational graft tolerance, interleukin-18 course, and results of retransplantation.

BACKGROUND: Posttransplant lymphoproliferative disorder (PTLD) can be resolved in many transplant patients by the reduction or cessation of immunosuppression, after which many grafts continue to function as the result of a form of operational tolerance. When graft function deteriorates, retransplantation may be an option. Cytokines such as interleukin (IL)-10 and IL-18 may play a role in PTLD tolerance induction and tumor regression. We report long-term follow-up on the duration of graft tolerance and the course of retransplantation in a series of patients who underwent kidney transplantation and demonstrated PTLD, and in whom we were able to perform IL-18 analyses. RESULTS: Patients were followed for up to 7 years after PTLD diagnosis. Treatment consisted of immunosuppression cessation with radiation therapy in cases with overt monomorphic lymphomas. All patients' PTLDs were resolved, and all patients but one (whose graft was removed) demonstrated a period of operational graft tolerance of up to 5 years. Five patients underwent retransplantation without sign of recurrence of the PTLD up to 3 years after transplantation. In the eight patients analyzed, IL-18 increased significantly during PTLD regression and follow-up in those with long-term operational tolerance. CONCLUSION: We report on a series of patients with resolved PTLDs demonstrating long-term recurrence-free survival, of whom most experienced a long period of operational graft tolerance. IL-18 seems to play a role in the resolution of the PTLDs. Five patients underwent retransplantation with standard immunosuppression without recurrence. A previous diagnosis of PTLD should not be regarded as a contraindication for later retransplantation.

Creatinine↗
PubMed2000

Cutaneous presentation of posttransplant lymphoproliferative disorder.

Posttransplant lymphoproliferative disorder (PTLD) is a known complication of solid organ and bone marrow transplantation. However, cutaneous manifestation is an extremely rare phenomenon of this disorder. We describe a lung transplantation patient with cutaneous PTLD who presented with bilateral extremity nodules and fever.

Female↗
PubMed1999

Chronic lymphoid leukemias and lymphoproliferative disorders.

Chronic lymphoproliferative disorders (CLPDs) are derived from the clonal proliferation of cytologically and immunophenotypically mature B or T cells. Although overlap is prominent, the CLPDs can generally be segregated into leukemias (predominant blood and bone marrow manifestations) and lymphomas (predominant extramedullary manifestations). This review discusses the leukemic processes, with emphasis placed on the blood and bone marrow features of these disorders. Many distinct types of B-cell and T-cell CLPDs have been described, and the classification of CLPDs requires the integration of morphologic features, immunophenotype, and clinical information; genotypic analyses might provide prognostic and biologic information. The various classification systems for the CLPDs will be presented, as well as a discussion of specific disease types, emphasizing those most commonly encountered in clinical practice.

Chronic Disease↗
PubMed1998

A clinically aggressive, polymorphic, polyclonal posttransplantation lymphoproliferative disorder.

Posttransplantation lymphoproliferative disorders (PTLD) have been divided, based on morphology, into polymorphic and monomorphic types, since findings in the literature reveal that polymorphic PTLD more likely responds to reduced immunosuppression. We report a clinically aggressive, polymorphic (and polyclonal) PTLD despite reduced immunosuppression and discuss possible therapeutic options. In addition, our case suggests that cytogenetic studies may be useful in determining prognosis when DNA molecular techniques do not detect monoclonality.

Aneuploidy↗
PubMed1997

Clinicopathologic features of posttransplant lymphoproliferative disorders.

Posttransplant lymphoproliferative disorders (PTLD) are primarily B lymphocyte tumors which are related to the Epstein-Barr virus. Recent studies have more clearly delineated neoplastic from hyperplastic forms of this disease. Factors associated with increased PTLD risk include recipient EBV seronegative status and heavy immunosuppression. The clinical presentation of PTLD is reviewed and lesser known features such as respiratory compromise or localization of tumors to skin are highlighted. The pathologic classifications of PTLD are surveyed and related to one another. Newer approaches to therapy, including the use of monoclonal antibodies and adoptive cellular immunotherapy are discussed.

Humans↗
PubMed2002

[Chronic lymphoproliferative disorders].

Chronic lymphoproliferative disorders include several entities that share an initial indolent presentation but are considered at present not to be curable. Follicular lymphoma are more frequent and well characterized; therapeutic recommendations are adapted to prognostic factors such as stage and tumour mass. Marginal zone lymphoma were more recently described and include extranodal MALT lymphomas as well as splenic, leukemic or nodal lymphomas, each with its own clinical features. Lymphocytic lymphomas resemble to chronic lymphocytic leukemia and can be treated in the same way, while mantle cell lymphomas have a very poor outcome. A better definition of these entities should allow to clarify therapeutic recommendations before more efficient treatments become available.

Diagnosis, Differential↗