SEARCH · PubMed Health

Results for “Lymphoproliferative Disorders”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 55 records · Page 3Linked to original sources

PubMed1988

Rearrangement of T-cell delta locus in lymphoproliferative disorders.

Studies of lymphoproliferative disorders using immunoglobulin and T-cell receptor genes have contributed to our understanding of clonality and lineages of these disorders. In this study, we examined the rearrangement of the recently discovered T-cell delta chain genes in a variety of lymphoproliferative diseases. We show here that six of 14 T-cell lymphomas and five of 23 B-cell lymphomas or B-cell leukemia cell lines have rearranged the delta loci, while two of two hyperimmune reactions retain germline configuration within these genes. Seven of ten cases of AILD were rearranged, and Lennert's lymphoma, which has been previously described as a T-cell malignancy, also contains rearrangements in the delta chain genes (three of five). Large cell anaplastic lymphomas positive for the activation antigen CD 30 also contain rearrangement in about one-half (five of 11) of the tumors examined. Two of seven of the Hodgkin's lymphomas studied contained a rearrangement for this gene. This study indicates that this newly identified T-cell delta gene is useful in evaluating clonality but is not lineage specific. However, with only one exception (in 28 rearrangements), this gene rearranges in tumors with gamma and beta chain gene rearrangements, indicating that when used in conjunction with the other TcR genes, delta rearrangement may also be useful in evaluating lineages.

B-Lymphocytes↗

PubMed1997

Posttransplantation lymphoproliferative disorder in cardiac transplant allografts.

Posttransplantation lymphoproliferative disorder occurs in 1.5% to 13% of heart transplant recipients and rarely involves the allograft. We report a case of posttransplantation lymphoproliferative disorder restricted to the mitral valve in a heart transplant recipient. Thirteen cases of cardiac allograft involvement by posttransplantation lymphoproliferative disorder are reported in the literature. None are restricted to the allograft. Five specify sites of cardiac involvement. Valvular masses without infection necessitate evaluation for posttransplantation lymphoproliferative disorder involvement of the cardiac allograft valve.

Heart Transplantation↗

PubMed1994

Lymphoproliferative disorders and other tumors complicating immunodeficiencies.

Lymphoproliferative disorders and selected carcinomas which occur as complications of primary or secondary immunodeficiencies are frequently fatal. The incidence rates of these cancers vary from 1% to as high as 25% among specific groups of persons with primary (genetically-determined) immunodeficiencies as well as acquired immunodeficiencies, including immunosuppressed organ transplant recipients and individuals infected with HIV. Lymphoproliferative disorders including Epstein Barr virus (EBV) associated B cell lymphoproliferative disease (BLPD) and Hodgkin's disease represent the predominant category of tumors in both primary and acquired immunodeficiencies. EBV is an important cofactor common to many, but not all, B cell "lymphomas." Immunodeficient individuals who are at risk for developing EBV BLPD may demonstrate both inadequate immune responses to the virus as well as generalized immunoregulatory dysfunction reflected as imbalances in cytokine production favoring the proliferation of transformed B lymphocytes. Historically, the success of treatment of lymphoproliferative disorders in immunodeficiencies with conventional multi agent chemotherapies and/or radiation has been limited by unfavorable tumor response rates and high morbidity and mortality related to intercurrent opportunistic infections. With improvements in supportive care and the use of recombinant biologic response modifiers such as alpha interferon and/or other immunotherapies to treat EBV BLPD, survival of immunodeficient hosts following tumor diagnosis may improve. In addition to lymphoproliferative disorders, patients with congenital immunodeficiencies associated with IgA deficiency (including ataxia telangiectasia and Common Variable Immunodeficiency) are at increased risk for gastrointestinal carcinomas. Early detection and surgical excision of such tumors can result in prolonged survival in such patients.

Humans↗

PubMed1990

Effects of interferon alpha on autocrine growth factor loops in B lymphoproliferative disorders.

The B lymphoproliferative disorders B chronic lymphocytic leukemia (B-CLL) and hairy cell leukemia (HCL) produce a number of autocrine growth factors, including tumor necrosis factor (TNF), interleukin 6 (IL-6), and IL-1, all of which may induce positive feedback growth loops. If such malignancies depend on these autocrine growth loops for survival, their interruption may be therapeutically valuable. Interferon alpha (IFN-alpha) abrogates TNF- or IL-6-induced proliferation of HCL and B-CLL cells in vitro and has therapeutic activity in these diseases. We have investigated the possibility that IFN-alpha may act by interrupting autocrine growth factor loops. If purified B-CLL or HCL cells are cultured in the presence of TNF, there is induction of mRNA for TNF, IL-1 alpha, IL-1 beta, and IL-6. However, culture in the presence of IFN-alpha in addition to TNF reduced the level of mRNA for all these cytokines, compared with cells cultured in TNF alone. While cytokine mRNA levels were diminished, levels of mRNA for the ribonuclease activator 2-5A synthetase were increased. Analysis of the kinetics of cytokine mRNA production showed that levels fall shortly after the rise of 2-5A synthetase mRNA. IFN-alpha may produce these effects by shortening the half-life of cytokine mRNA, since TNF mRNA half-life in B-CLL and HCL cells is substantially reduced when the cells are cultured with IFN-alpha. These data suggest that IFN-alpha may mediate its therapeutic effects in these malignancies by blocking autocrine growth factor loops.

Adult↗

PubMed1996

BCL-2 but not its Epstein-Barr virus-encoded homologue, BHRF1, is commonly expressed in posttransplantation lymphoproliferative disorders.

Posttransplantation lymphoproliferative disease (PTLD) is virtually always associated with Epstein-Barr virus (EBV) infection. BCL-2 and other proteins that confer resistance to apoptosis have been implicated in the pathogenesis of a variety of malignancies including lymphomas. One EBV protein, BHRF1, is a homologue of BCL-2, whereas another, the latency membrane protein 1 (LMP-1), upregulates BCL-2 expression in vitro. In the present study, we used immunohistochemistry to study the expression of these viral and cellular proteins as well as a variety of other EBV-encoded proteins in PTLD. BHRF1 was not detected in any PTLD specimen, whereas BCL-2 was shown in 12 of 17 lesions examined. With one exception, all LMP1-positive cases also expressed BCL-2 and the absence of LMP1 was always associated with a lack of BCL-2 expression. The results do not support a role for the EBV homologue of BCL-2 in PTLD, but they do support a role for viral induction of BCL-2 expression.

Amino Acid Sequence↗

PubMed1994

Patterns of Epstein-Barr virus latent and replicative gene expression in Epstein-Barr virus B cell lymphoproliferative disorders after organ transplantation.

B cell lymphoproliferative disorders arising in organ transplant recipients (B cell posttransplant lymphoproliferative disorders [PTLD]) are generally associated with EBV. In previous reports, B cell PTLD were shown to express the full pattern of EBV latent genes, as in vitro-established lymphoblastoid cell lines. Although viral linear DNA was detected in 40% of lymphoproliferative disorders from immunocompromised hosts, immunophenotypic studies failed to detect late EBV replicative antigens. The aim of this study was to investigate the relationship of EBV latent gene expression in B cell PTLD to morphology, clonality, and immunophenotype, and to examine the replicative state of EBV in malignant cells. For this purpose, 9 cases of EBV-related B cell PTLD were analyzed. Immunoglobulin gene rearrangements were detected by Southern blot analysis. The presence of EBV was assessed by Southern blot and by in situ hybridization. B cell differentiation antigens, adhesion and activation molecules, and EBV latent and replicative gene expression were studied using immunohistochemistry techniques. We demonstrated that EBV-related B cell PTLD exhibited varying patterns of latent viral gene expression. Higher levels of adhesion molecules were detected in latent membrane protein 1 (LMP1) or LMP1 plus EBV nuclear antigen 2 (EBNA2)-positive tumors than in LMP1 and EBNA2-negative tumors. In contrast, there was no relationship between CD21 and CD23 expression and latent EBV phenotype. Activation of the EBV replicative cycle was highlighted by BamHI Z left frame 1 expression in 5 of 9 cases. Less frequent expression of late viral proteins suggested that the initiation of the EBV lytic cycle might not always lead to virions production.

Adult↗

PubMed1994

The spectrum of chronic lymphoproliferative disorders in Chinese people. An analysis of 64 cases.

BACKGROUND: Chronic lymphoproliferative disorders are considered rare in Oriental patients and are thought to constitute only 2% of all leukemias in these patients, compared to 20-30% in Western patients. We conducted a retrospective analysis of Chinese patients with chronic lymphoproliferative disorders to define the frequency and spectrum of these disorders. METHODS: A consecutive series of Chinese patients with leukemia and lymphoproliferative disorders seen at two regional hospitals in Hong Kong were analyzed retrospectively. The diagnosis of chronic lymphoproliferative disorders was based on morphologic and immunologic criteria proposed by the French-American-British Cooperative Study Group. RESULTS: Sixty-four Chinese patients with chronic lymphoproliferative disorders were identified, and these patients constituted 19% of a total of 342 cases of leukemia diagnosed in 3 years. Chronic lymphocytic leukemia was the most common form, occurring at a frequency of 12.5% of all leukemias. The clinicopathologic features of these patients were similar to those of Western patients, except that Chinese patients tended to present with more advanced (Rai's Stages III and IV; Binet's Stage C) and bulky (splenomegaly > 9 cm) disease, and expressed lambda light chain about six times more frequently. Other chronic lymphoproliferative disorders identified in this study included prolymphocytic leukemia, mantle zone lymphoma, hairy cell leukemia, splenic lymphoma with villous lymphocytes, large granular lymphocyte leukemia, and Sezary syndrome. The authors did not identify any case of human T-cell lymphotropic virus-I-related lymphoproliferative disorders within the study period. CONCLUSION: In addition to providing the frequencies of various chronic lymphoproliferative disorders in southern Chinese people, this study also showed that these disorders no longer should be considered rare in this population. Inherent biologic differences between lymphoproliferative disorders in Chinese and Western patients also may exist.

Adult↗

PubMed2005

Gastrointestinal involvement of posttransplant lymphoproliferative disorder in lung transplant recipients: report of a case.

PURPOSE: Lymphoproliferative disorder is a well-recognized complication of lung transplantation. Risk factors include Epstein-Barr virus infection and immunosuppression. The gastrointestinal manifestations of post-transplant lymphoproliferative disorder in lung transplant recipients have not been fully characterized. METHODS: Case presentation and 16 previously reported cases of post-transplant lymphoproliferative disorder with gastrointestinal involvement are reviewed. RESULTS: Patient ages ranged from 25 to 65 (median, 52) years. Median time from lung transplantation to onset of posttransplant lymphoproliferative disorder was 36 (range, 1-109) months; 35 percent of cases (6/17) occurred within 18 months; Eighty-eight percent of patients (15/17) had positive Epstein-Barr virus serology before transplantation. In five patients (29 percent), the posttransplant lymphoproliferative disorder also involved sites other than the gastrointestinal tract. The most common gastrointestinal site of posttransplant lymphoproliferative disorder was the colon, followed by the small intestine and stomach. Clinical features included abdominal pain, nausea, and bloody diarrhea. Diagnosis was based on typical pathologic changes on gastrointestinal tract biopsy obtained mainly by colonoscopy. Treatment included a reduction in the immunosuppressive regimen in 15 of 17 cases (88 percent) and surgical resection in 10 (59 percent). One patient was untreated. Seven of 16 patients (44 percent) responded to treatment and 9 patients died. Median time from onset of posttransplant lymphoproliferative disorder to death was 70 (range, 10-85) days. CONCLUSIONS: Posttransplant lymphoproliferative disorder with gastrointestinal involvement is a unique entity that should be considered in all Epstein-Barr-Virus-positive lung transplant recipients who present with abdominal symptoms. Although immunosuppressive modulation and resection can lead to remission, the risk of death is 50 percent.

Aged↗

PubMed1998

The molecular genetics of post-transplantation lymphoproliferative disorders.

The post-transplantation lymphoproliferative disorders represent a significant clinical and diagnostic problem. However, these disorders also represent an important biological model for studying the development and progression of lymphoid neoplasia in immune deficiency. Accurate diagnosis and classification of these disorders requires correlative multiparametric analysis of the clinical behavior of the patient with the histopathological features, immunophenotype, clonal composition, and genetic alterations of the lymphoproliferative disorder. Such analyses should also assist in furthering our understanding of the pathogenesis of these disorders.

Blotting, Southern↗

PubMed1994

2-Chlorodeoxyadenosine therapy in patients with T-cell lymphoproliferative disorders.

Mature T-cell lymphoproliferative disorders comprise a heterogenous group of diseases for which there is no standard therapy. These disorders are uncommon, and are usually treated similarly to their B-cell counterparts, but with less success. Nucleoside analogues have proven effective in indolent B-cell disorders but have been less well explored in T-cell malignancies. We treated 22 patients with mature T-cell lymphoproliferative diseases with 2-chlorodeoxyadenosine (2-CDA) administered as a continuous infusion at a daily dose of 4 mg/m2 over 7 days. Nineteen of the patients had received prior therapy with a median number of prior regimens of three. Eleven patients had leukemia or large granular lymphocytosis, eight patients had mycosis fungoides, and three had T-cell lymphoma. Nine patients (41%) responded to 2-CDA. Four of the patients had responses that were complete remissions, and three of these four patients remain in remission at 23, 24, and 23 months. The only important toxic effects were fever or infection, seen during 38% of courses. In conclusion, 2-CDA appears to be an effective therapy in T-cell lymphoproliferative disorders and deserves wider evaluation in this subset of patients.

Adult↗

PubMed1996

Benign lymphoproliferative disorders.

OBJECTIVE: To provide a review of three benign lymphoproliferative disorders commonly encountered in nursing practice: (1) infectious mononucleosis, (2) cat scratch disease, and (3) sarcoidosis. DATA SOURCES: Research studies, review articles, and book chapters pertaining to benign lymphoproliferative disorders. CONCLUSIONS: Benign lymphoproliferative disorders may be associated with infections, autoimmune disorders, hypersensitivity reactions, and unknown causes. Most of these disorders are self-limiting; however, some are associated with significant morbidity and mortality. IMPLICATIONS FOR NURSING PRACTICE: Benign as well as malignant lymphoproliferative disorders will be encountered with increasing frequency by nurses in ambulatory settings. Nurses need to have a basic knowledge of these disorders that present a diagnostic and management challenge.

Adult↗

PubMed2004

Flow cytometric evaluation of posttransplant B-cell lymphoproliferative disorders.

CONTEXT: Posttransplant B-cell lymphoproliferative disorders (PTLDs) constitute a heterogeneous group that includes hyperplastic and unique polymorphic lesions at one end of the spectrum and monomorphic lymphoid proliferations indistinguishable morphologically from conventional B-cell non-Hodgkin lymphomas (NHLs) at the other end. Almost all the PTLDs are of B-cell origin, with only rare examples of T-cell phenotype described. Despite a plethora of information available on the morphologic spectrum, pathogenetic role of Epstein-Barr virus, and various treatment options, a detailed flow cytometric immunophenotypic evaluation of PTLDs is largely lacking. OBJECTIVE: To evaluate the immunophenotypic profiles of various PTLDs using multiparameter flow cytometric analysis to compare and contrast with conventional de novo B-cell lymphoproliferative disorders and to identify any immunophenotypic patterns useful in diagnosis. DESIGN: We retrospectively analyzed data on the immunophenotype of 25 cases of pediatric and adult PTLD (12 cases of monomorphic PTLD [m-PTLD] and 13 cases of polymorphic PTLD [p-PTLD]) using multiparameter flow cytometry in addition to routine morphologic and immunohistochemical evaluation. The flow cytometric immunophenotypic data were also compared and contrasted with 334 cases of various de novo B-cell NHLs during the same period as a control group. RESULTS: We observed a much higher incidence of lack of surface immunoglobulin light chains and CD20 expression in B-cell PTLDs using multiparameter flow cytometry in comparison with de novo B-cell NHL as a group (with the exception of small lymphocytic lymphoma). Four (16%) of 25 cases of PTLD (3 m-PTLD and 1 p-PTLD) showed almost complete lack (CD20%/CD19% ratio < 1:9) of CD20 expression in contrast to only 8 ( approximately 2%) of 334 cases of de novo B-cell NHL (P =.007). Several other cases of both m-PTLD and p-PTLD also showed partial and dim expression of CD20. Nine (36%) of 25 cases, including 5 cases of m-PTLD and 4 of p-PTLD, showed either an almost complete lack (light chains%/CD19% ratio < 1:9) or significant loss (>50% loss) of surface immunoglobulin light chains in contrast to less than 5% incidence of light-chain negativity in conventional de novo B-cell NHL. Immunoglobulin light-chain clonality was observed in 9 cases (5 m-PTLD and 4 p-PTLD). Seven cases (5 p-PTLD and 2 m-PTLD) had polyclonal expression of immunoglobulin kappa and lambda light chains. The m-PTLD showed expression patterns of CD5, CD10, and CD23 similar to their de novo counterparts. CONCLUSIONS: Both polymorphic and monomorphic PTLDs show a higher incidence of lack of CD20 and surface immunoglobulin light-chain expression. The lack of CD20 expression in these lesions may have therapeutic implications, since anti-CD20 antibody has increasingly become an important modality in the treatment of B-cell lymphoproliferative disorders, including posttransplant disorders.

Adolescent↗

PubMed2005

Novel treatment with rituximab of oropharyngeal posttransplant lymphoproliferative disorder after heart transplantation.

Posttransplant lymphoproliferative disorders are severe complications that arise after solid organ transplantation, which are often related to Epstein- Barr virus. Reports are anecdotal, and a standardized therapy does not exist. We report a case of a 36-year-old man who developed posttransplant lymphoproliferative disorder of the oropharynx 1 year after receiving a heart transplant. A short review of the literature is presented, after which a new therapeutic approach that combines antiviral therapy, monoclonal antibodies, and a sirolimus-based maintenance immunosuppression regimen with reduced target trough levels of tacrolimus is introduced. The patient achieved complete remission and was free from recurrence 18 months after the therapy was initiated.

Adult↗

PubMed2002

Initial spontaneous remission of posttransplantation Epstein Barr virus-related B-cell lymphoproliferative disorder of the skin in a renal transplant recipient: case report and review of the literature on cutaneous B-cell posttransplantation lymphoproliferative disease.

Primary cutaneous posttransplantation B-cell lymphoproliferative disorder is rare. The few previously reported patients were all treated with surgery, radiotherapy, or lowering of immunosuppression. We describe a 65-year-old woman presenting with an intermammary skin ulcer 21 years after renal transplantation, proving on biopsy to be an Epstein Barr virus (EBV)-related posttransplantation B-cell lymphoproliferative disorder. A few weeks later, the skin ulcer showed complete clinical regression. Hematologic staging evaluation showed no evidence of extracutaneous involvement. Despite continuation of immunosuppression, the patient stayed free of disease until 18 months after initial diagnosis, when she developed a progressive hemiparesis and died of acute myocardial infarction. At autopsy, a recurrent B-cell posttransplantation lymphoproliferative disorder in the left side of the thalamus region (measuring 1 x 0.8 cm) was established. The long interval between the primary cutaneous lesion and the localized brain recurrence supports primary skin posttransplantation lymphoproliferative disorder, especially because the patient was not treated for her posttransplantation lymphoproliferative disorder. Review of the literature on primary cutaneous posttransplantation B-cell lymphoproliferative disorder and this case gives the impression that cutaneous posttransplantation B-cell lymphoproliferative disorders of B-cell lineage behave in a more benign manner than identical lesions arising extracutaneously. Because of the rare occurrence of posttransplantation B-cell lymphoproliferative disorder primarily involving the skin, extracutaneous origin should be excluded. If B-cell lineage can be established, EBV is present, alterations in oncogenes or tumor suppressor genes associated with malignant lymphoma are absent, and bcl-6 gene mutation associated with progression is absent, initially aggressive treatment might be avoided. However, long-term clinical follow-up with prolonged maintenance therapy (reduction of immunosuppression or antiviral therapy) for prevention of recurrent posttransplantation lymphoproliferative disorder seems indicated, as is demonstrated by the case reported in the current study.

Aged↗

PubMed2006

Occurrence of post-transplant lymphoproliferative disorders among over thousand adult recipients: any role for hepatitis C infection?

BACKGROUND: Post-transplant lymphoproliferative disorders represent an increasingly important complication of organ transplantation. Although the majority of the post-transplant lymphoproliferative disorder are etiologically related to Epstein-Barr virus infection other factors may play a role. Hepatitis C virus may induce clonal expansion of B-lymphocytes and has been associated with extrahepatic lymphoproliferative disorders. OBJECTIVES: In this study, we have evaluated: (i) the prevalence of post-transplant lymphoproliferative disorder; (ii) presence of Epstein-Barr virus in post-transplant lymphoproliferative disorder tissue; and (iii) the potential association between post-transplant lymphoproliferative disorder development and hepatitis C virus infection in a large cohort of adult solid organ transplant recipients. METHODS: The study involved 1011 liver, heart and kidney-transplanted patients. Different immunosuppression therapy was recorded from all patients, all were screened for hepatitis C virus infection. When post-transplant lymphoproliferative disorder developed, Epstein-Barr virus encoded RNA by in-situ hybridization and EBNA-1 and gp220 by polymerase chain reaction was assessed in tissue samples. RESULTS: The overall prevalence of post-transplant lymphoproliferative disorder was 1.4% (2.5% in heart, 0.9% in liver and 0.8% in kidney-transplanted patients) and significantly higher in hepatitis C virus positive than in hepatitis C virus negative patients (3.6 % vs 1.2 %; P=0.04). Epstein-Barr virus was present in 10 (77%) out of 13 tumors tested. Two out of three Epstein-Barr virus-negative post-transplant lymphoproliferative disorder developed in hepatitis C virus-positive patients. Thirteen out of 15 (86%) post-transplant lymphoproliferative disorder patients had undergone antithymocyte globulin/OKT3 induction therapy. CONCLUSIONS: Epstein-Barr virus, induction immunosuppression, rejection therapy and also hepatitis C virus infection may play a role in the multifactorial pathogenesis of post-transplant lymphoproliferative disorder.

Adult↗

PubMed1994

Autoimmune thrombocytopenic purpura complicating lymphoproliferative disorders.

Thrombocytopenia in patients with lymphoproliferative disorders is usually multifactorial. In some patients, peripheral destruction of platelets by platelet autoantibodies may account in part for the thrombocytopenia. However, the diagnosis of autoimmune thrombocytopenic purpura in this group of patients can be difficult due to the splenomegaly and compromised bone marrows in some of these patients. The development of autoimmune thrombocytopenic purpura in these patients does not affect the eventual outcome of the underlying lymphoproliferative disorders. Unfortunately the current available therapy for this condition is unsatisfactory. Other innovative treatment modalities are therefore much needed.

Adult↗

PubMed2006

Posttransplant lymphoproliferative disorder: a pictorial review.

Posttransplant lymphoproliferative disorder (PTLD) is a serious and potentially fatal complication after solid organ and haemopoietic stem cell transplantation. The frequency of PTLD varies with the type of organ transplant but overall it affects 2-10% of all solid organ transplant recipients. Most cases develop within 1 year after the transplant, although occasional cases present 5-10 years later. Posttransplant lymphoproliferative disorder is clinically and pathologically heterogeneous - the majority are of the non-Hodgkin's lymphoma type, whereas Hodgkin's lymphoma arising after transplantation is rare. We have retrospectively reviewed patients with a histological diagnosis of PTLD after a solid organ transplant. We present the imaging features and a clinical review of this condition. Early diagnosis of PTLD may alter the management and outcome of the disease. The radiologist can play a vital role in establishing the diagnosis by imaging features supplemented with percutaneous biopsy and also in monitoring the disease response to treatment.

Humans↗

PubMed1998

Combined radiation and chemotherapy in posttransplant lymphoproliferative disorder.

The optimal treatment for posttransplant lymphoproliferative disorder which has progressed despite a reduction in immunosuppression has not been defined. We report on two patients with stage I posttransplant lymphoproliferative disorder who developed progressive disease despite a reduction in the level of immunosuppression. Both patients were treated with combined short course CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy followed by involved-field radiation therapy. In both patients, a rapid response was obtained followed by complete remission. Combined modality therapy can be utilized successfully in progressive limited stage posttransplant lymphoproliferative disorder.

Antineoplastic Combined Chemotherapy Protocols↗