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 181 records · Page 10Linked to original sources

PubMedDate not supplied

Epstein-Barr virus-associated leiomyomatosis and posttransplant lymphoproliferative disorder in a child with severe combined immunodeficiency: case report and review of the literature.

The occurrence of smooth muscle neoplasms and lymphoproliferative disorders in immunocompromised patients is well recognized. We report the case of an 8-year-old girl with adenosine deaminase (ADA)-deficient severe combined immunodeficiency (SCID) status post-bone marrow transplant (BMT), in whom Epstein-Barr virus (EBV) was detected in innumerable leiomyomas involving the gallbladder (leiomyomatosis), and multifocal leiomyomas in liver, spleen, pancreas, intestinal tract, and lung. The leiomyomas of the gallbladder, liver, spleen, and lung were asymptomatic, while those located in the colon became clinically manifest by recurrent lower intestinal hemorrhage. The patient also developed extensive EBV-associated polymorphic lymphoproliferative disorder (PTLD) in nodal and extranodal sites. In addition, there were pulmonary and gastric adenovirus and small and large intestine cryptosporidum infections. Our case appears to be the first example of leiomyomatosis of the gallbladder coexisting with multifocal leiomyomas of the liver, spleen, pancreas, intestinal tract, and lung, as well as EBV-derived lymphoproliferative disorder in a young girl with ADA-deficient SCID. Awareness of the pattern of involvement and of the coexistence of benign leiomyomatous proliferations with lymphoproliferative disorder is of value when gallbladder, pancreatic, biliary tree, lung, and intestinal lesions become clinically manifest in these patients. The demonstration of EBV infection in both leiomyomata and the PTLD suggests a common pathogenesis that may have therapeutic and prognostic implications.

Adenosine Deaminase↗

PubMed2002

KSHV- and EBV-associated germinotropic lymphoproliferative disorder.

Kaposi sarcoma-associated herpesvirus (KSHV) is known to be associated with 3 distinct lymphoproliferative disorders: primary effusion lymphoma (PEL), multicentric Castleman disease (MCD), and MCD-associated plasmablastic lymphoma. We report 3 cases of a previously undescribed KSHV-associated lymphoproliferative disorder. The disease presented as localized lymphadenopathy and showed a favorable response to chemotherapy or radiotherapy. Histologically, the lymphoproliferation is characterized by plasmablasts that preferentially involved germinal centers of the lymphoid follicles, forming confluent aggregates. They were negative for CD20, CD27, CD79a, CD138, BCL6, and CD10 but showed monotypic kappa or lambda light chain. Clusters of CD10(+)CD20(+) residual follicle center cells were identified in some of the follicles. The plasmablasts were positive for both KSHV and EBV, and most of them also expressed viral interleukin-6 (vIL-6). Unexpectedly, molecular analysis of whole tissue sections or microdissected KSHV-positive aggregates demonstrated a polyclonal or oligoclonal pattern of immunoglobulin (Ig) gene rearrangement. The plasmablasts showed somatic mutation and intraclonal variation in the rearranged Ig genes, and one case expressed switched Ig heavy chain (IgA), suggesting that they originated from germinal center B cells. We propose calling this distinctive entity "KSHV-associated germinotropic lymphoproliferative disorder."

Antineoplastic Combined Chemotherapy Protocols↗

PubMed2000

Use of radiation therapy in posttransplant lymphoproliferative disorder (PTLD) after liver transplantation.

Posttransplant lymphoproliferative disorder (PTLD) is a common and life-threatening complication of immunosuppression used to prevent rejection of solid organ and bone marrow transplants. There is no standardized treatment algorithm, but numerous management strategies are available. We describe a patient who developed a solitary lymphoproliferative lesion in the porta hepatis 9 months after orthotopic liver transplant. Following reduction in immunosuppression with no response, she was treated with involved field radiotherapy utilizing CT-based treatment planning. A partial radiographic response was obtained, and she has not developed disease in the engrafted liver or systemically. Based on the present case report, involved field radiotherapy seems to be a reasonable treatment option for patients with localized PTLD. Int. J. Cancer (Radiat. Oncol. Invest.) 90:104-109, 2000.

Algorithms↗

PubMed1991

The expression of CD22 (Leu 14) and CD11c (LeuM5) in chronic lymphoproliferative disorders using two-color flow cytometric analysis.

The monoclonal antibodies (MoAbs) CD22 and CD11c recognize B-lymphocyte- and monocyte-associated antigens, respectively. Reports indicate that when these two MoAbs co-express, they represent a unique marker for hairy cell leukemia (HCL) although neither is specific for that disease. The authors evaluated the expression and diagnostic utility of CD22 and CD11C in specimens from 26 normal subjects, 29 patients, with various nonlymphoproliferative disorders (NLPDs), and 75 patients with different types of chronic lymphoproliferative disorders (CLDs) using two-color flow cytometric analysis of peripheral blood lymphocytes. Lymphocytes co-expressed CD22 and CD11c in less than or equal to 3% of the normal subjects and in less than or equal to 6% of the patients with NLPDs. These markers were expressed in greater than 10% of the lymphocytes of 46% (32/69) of the patients with B-cell CLDs: B-cell chronic-lymphocytic leukemia, 9/41; B-cell non-Hodgkin's lymphoma, 8/14; HCL, 11/11; B-cell lymphoproliferative disorder (NOS), 1/2; and B-cell prolymphocytic leukemia, 1/1. None (0/6) of the lymphocytes of patients with T-cell CLDs expressed greater than 10% CD22-positive (CD22+) or CD11c-positive (CD11c+) cells. The HCL cases demonstrated a unique CD22+CD11c+ fluorescence histogram pattern, distinct from other lymphoproliferative disorders, that was characterized by uniformly intense CD11c and CD22 fluorescence. Differences in the expression of the CD22+CD11C- and CD22+CD11C+ phenotypes between diagnostic groups were found, most notable was a paucity of CD22+CD11c+ cells in lymphocytes of patients with HCL. CD22 also had more variable expression than CD19 and HLA-DR in the cases of B-cell CLD. This study demonstrates that the CD22+CD11c+ phenotype is not unique to HCL but is a consistent feature of that disorder and that the immunofluorescence pattern of co-expression in HCL is diagnostically useful.

Antigens, CD↗

PubMed1994

Lymphoproliferative disorders in non-AIDS-associated Kaposi's sarcoma. The Johannesburg Hospital experience, 1980-1992.

The association of the non-AIDS-related, classic form of Kaposi's sarcoma (KS) with secondary malignancies, especially lymphoproliferative disorders, has frequently been noted. However, in endemic African-type KS, such an association has been reported only rarely. A review of 62 non-AIDS-related cases of KS treated and followed up at Johannesburg General Hospital between 1980 and 1992 revealed 8 patients (13%) in whom KS was associated with malignant lymphoproliferative disorders. The prevalence of secondary lymphoproliferative disorders was not significantly different among patients with classic KS (3/15; 20%) when compared with those who had African KS (4/47; 8%). In both forms of KS subtle disturbances of immunity have been described which may play a role in the pathogenesis of secondary lymphoproliferative disorders, although the factors responsible and the pathogenetic mechanisms involved in malignant lymphoid transformation in these patients have not been fully elucidated.

Adult↗

PubMed1987

B-cell lymphoproliferative disorders in children after bone marrow transplantation: radiologic manifestations.

The radiographic findings in five pediatric patients in whom unregulated B-cell lymphoproliferative disorders developed following bone marrow transplantation are described. Four patients received T-cell-depleted bone marrow from mismatched donors and one received nondepleted marrow from a matched sibling donor. These disorders are similar to B-cell lymphoproliferative disorders that have been described in other immunosuppressed hosts. They are associated with Epstein-Barr virus and range from polyclonal proliferation without cytogenetic abnormalities to monoclonal lymphoma with clonal cytogenetic changes. Unlike other postallograft lymphoproliferative processes, B-cell lymphoproliferative disorders in these patients have not responded to antiviral therapy, immunologic therapy, or chemotherapy. The radiographic patterns of disease include diffuse or focal hepatic involvement; gallbladder wall thickening; and pulmonary, soft-tissue, and basal-ganglion masses. These radiologic findings are not specific and evaluation of tissue histology is required for diagnosis.

Adolescent↗

PubMed2002

Successful treatment with rituximab of lymphoproliferative disorder in a child after cardiac transplantation.

Post-transplantation lymphoproliferative disorders (PTLDs) are life-threatening complications. We report the case of a 7-year-old girl in whom a lymphoproliferative disorder developed more than 2 years after cardiac transplantation. The patient was taking ganciclovir for Epstein-Barr virus hepatitis at the time the PTLD occurred. Rituximab, an anti-CD20 monoclonal antibody, given in the presence of reduced immunosuppression therapy, resulted in a complete response of the PTLD. The Epstein-Barr viral load in the peripheral blood, which was extremely high at diagnosis, dropped promptly and remained below the detection threshold 11 months after completion of therapy. We observed complete depletion of B lymphocytes until 7 months after rituximab therapy, which was associated with an important decrease in immunoglobulin levels.

Antibodies, Monoclonal↗

PubMed1991

CD3+, CD4-, CD8- large granular T-cell lymphoproliferative disorder.

Large granular T-cell lymphoproliferative disorder (LGTLD) is a heterogeneous disorder covering a broad spectrum of diseases and requiring further subdivision. Most reported cases emphasized its suppressor phenotype (T gamma cell or CD8+), but we encountered two cases of CD3+, CD4-, CD8- LGTLD. Both cases had a benign clinical course and required no chemotherapy despite persistent lymphocytosis. This unique phenotype has been reported in a few cases of acute lymphoblastic leukemia expressing the T-cell receptor (TcR) gamma chain gene and is considered the counterpart of thymocytes at the intermediate stage between early precursors and mature thymocytes. Our case 1 provides further evidence that the CD3+, CD4-, CD8- phenotype, indeed, expresses the TcR gamma chain gene. However, the negative reaction to terminal deoxynucleotidyl transferase in our case 1 indicates that this phenotype represents proliferation of peripheral T-cells, in which about 2% bear the CD3+, CD4-, CD8- phenotype in the normal population. The selective use of CD3, CD4, CD8, HNK-1 monoclonal antibodies and of cytochemical stains (acid phosphatase and alpha-naphthyl butyrate esterase) for characterization of this disorder is discussed.

Adult↗

PubMed1995

Fatal Epstein-Barr virus-associated lymphoproliferative disorder in childhood.

OBJECTIVE: We report five childhood cases of fatal Epstein-Barr virus-associated lymphoproliferative disorder to determine their immunopathologic and immunogenotypic features. DESIGN: Clinicopathologic features are described, using clinical, histologic, immunophenotypic, and genotypic examinations. SETTING: Autopsy cases were performed at Nagoya and Tsukuba university hospitals, Japan. RESULTS: Infiltrating lymphocytes, which were positive for the EBV genome by in situ hybridization, were polymorphic and showed polyclonal immunoglobulin staining in all five cases. Two cases, however, showed genetic clonality of EBV termini. In one case, the clonality was observed only in the spleen, and in the other case, clonal rearrangement of the JH gene was also found. The former case showed none of the morphologic features of neoplasia and presented a morphology similar to virus-associated hemophagocytic syndrome. In the latter case, infiltrating lymphoid cells were much less polymorphous, and nodular masses of the lymphoid cells in colon and lung specimens were observed. CONCLUSION: We suggest that some polyclonal EBV-associated lymphoproliferative disorders develop into polymorphic, but genotypically monoclonal, lymphoproliferative disorders.

Adolescent↗

PubMed1998

Epstein-Barr virus-associated lymphoproliferative disorder after autologous bone marrow transplantation: report of two cases.

Epstein-Barr virus-associated lymphoproliferative disorders have been frequently reported as a complication of solid organ and allogeneic bone marrow transplantation. Their occurrence is rare after autologous bone marrow transplantation (BMT) with only five published reports in the literature. We report two cases of post-transplant lymphoproliferative disorder occurring after autologous BMT for Hodgkin's disease and non-Hodgkin's lymphoma. Post-transplant lymphoproliferative disorders can occur after autologous BMT and should be included in the differential diagnosis of patients with persistent fever, adenopathy or pulmonary infiltrates.

Adult↗

PubMedDate not supplied

Intrathoracic lymphoproliferative disorders and lymphoma.

The spectrum of lymphoproliferative disorders is truly a continuum from benign diseases to malignant aggressive lymphomas. Remarkable advances in laboratory techniques of monoclonal antibody production and molecular biology over the last decade allow more sophisticated analysis of these diseases. It is now apparent that some entities previously labeled "benign" are actually non-Hodgkin's lymphomas. We review the current knowledge of this spectrum including the histopathologic and clinical findings, as well as the radiographic characteristics. Understanding the diseases, treatment challenges, and follow-up requirements aids the radiologist in a more efficient and cost-effective evaluation of these patients.

Adolescent↗

PubMed2005

Successful laser ablation of diffuse laryngeal swelling in posttransplant lymphoproliferative disorder: the Japanese first case.

Posttransplant lymphoproliferative disorder (PTLD) is a well-known complication of pediatric organ transplantation. The first symptoms are often in the ear, nose, or throat (ENT) area. We report the first Japanese case of an airway obstruction in PTLD. A 7-year-old boy who had a liver transplant at the age of 2 years presented with progressive stridor. Fiberoptic examination revealed enlarged epiglottis and swelling arytenoids. Highly suspicious of early-PTLD, he was treated with reduction of immunosuppression, but no improvement on his larynx was observed. Laser ablation of swelling arytenoids worked. Surgical debulking is effective to ease an airway obstruction in cases of PTLD.

Airway Obstruction↗

PubMed2003

Post-transplant lymphoproliferative disorder: a review.

Post-transplant lymphoproliferative disorder (PTLD) represents a spectrum of Epstein-Barr virus-related (EBV) clinical diseases, from a benign mononucleosis-like illness to a fulminant non-Hodgkin's lymphoma. In the setting of hematopoietic stem cell transplantation, PTLD is an often-fatal complication occurring relatively early after transplant. Risk factors for the development of PTLD are well established, and include HLA-mismatching, T-cell depletion, and the use of antilymphocyte antibodies as conditioning or treatment of graft-versus-host disease. Early recognition of PTLD is particularly important in the SCT setting, because PTLD in these patients tends to be rapidly progressive. Familiarity with the clinical features of PTLD and a heightened level of suspicion are critical for making the diagnosis. Surveillance techniques with EBV antibody titers and/or polymerase chain reaction (PCR) may have a role in some high-risk settings. Immune-based therapies such as monoclonal anti-B-cell antibodies, interferon-alpha, and EBV-specific donor T cells, either as treatment for PTLD or as prophylaxis in high-risk patients, represent promising new directions in the treatment of this disease.

Histocompatibility Testing↗

PubMed1997

Remission of posttransplant lymphoproliferative disorder after interferon alfa therapy.

Posttransplant lymphoproliferative disorder (PTLD) is one of the major complications of immunosuppressive therapy. PTLD is strongly associated with the Epstein-Barr virus (EBV). It is believed that EBV-infected B cells proliferate in an unchecked manner due to suppression of cytotoxic T cells and elevation of B cell-promoting cytokines. There is no consensus on the treatment of PTLD other than reduction of immunosuppressive therapy. We report a case of PTLD with monoclonal B cells confined to the lymph nodes. The patient did not initially respond to reduction of immunosuppression, oral acyclovir, and intravenous immunoglobulin injection. She subsequently responded when subcutaneous injections of interferon alfa (5 million U) were given three times a week. The patient received a 3-mo course of interferon and remained in remission 12 mo after treatment. Her graft function was well maintained, and cyclosporin A was restarted 2 mo after achieving remission. The clinical manifestations, risk factors, pathogenesis, and treatment of PTLD, as well as 12 previously reported cases of PTLD treated with interferon, were reviewed. On the basis of the results presented here, it appears that interferon alfa may be useful in treating PTLD and that there is a need for further clinical trials.

Aged↗

PubMed2001

Successful outcome with a "quintuple approach" of posttransplant lymphoproliferative disorder.

BACKGROUND: The treatment of posttransplant lymphoproliferative disorder (PTLD) remains empirical. We review our treatment of seven cases of PTLD consisting of five interventions: 1) reduction of immunosuppression; 2) antiviral drugs; 3) interferon-alpha; 4) gamma-globulins; and 5) anti-CD19 monoclonal antibodies. METHODS AND RESULTS; Seven consecutive patients who had undergone a simultaneous pancreas-kidney, liver, heart, or kidney transplantation were treated. One patient acquired a primary EBV infection with an oligoclonal immunoblastic lymphoma early after pancreas-kidney transplantation; all others developed a monoclonal polymorphic or immunoblastic lymphoma 2 to 123 months after transplantation. In all patients extranodal sites were involved, in three the graft was also involved. Five patients received the full quintuple approach and all rapidly obtained a complete remission (CR) with a median follow-up of 31 months (7-74 months). Of the two patients who did not receive interferon-alpha for fear of graft rejection one responded slowly with a CR after 7 months, and the other obtained a rapid CR followed by a relapse at 4 months. All three patients with a liver or heart transplant could keep their graft. All patients are still alive with a median follow-up of 31 months (7-74 months). CONCLUSION: This combined approach resulted in a favorable outcome in patients with high risk monoclonal PTLD after solid organ transplantation.

Acyclovir↗

PubMed2002

Fascin expression in CD30-positive cutaneous lymphoproliferative disorders.

BACKGROUND: CD30-positive cutaneous lymphoproliferative disorders (LPD) represent a spectrum of diseases ranging from low-grade (lymphomatoid papulosis; LyP) to high-grade (pleomorphic and anaplastic large-cell lymphoma; PTL, ALCL) with overlapping morphologic and immunophenotypic features. The common phenotypic hallmark is the expression of CD30-antigen by the tumor cells which morphologically resemble Reed-Sternberg cells. Although LyP is a non-fatal recurring disorder, it is associated with systemic lymphomas including Hodgkin's lymphoma (HL), mycosis fungoides (MF) and ALCL in 5-20% of the cases. Currently there is no marker to predict the development of systemic lymphomas in patients with LyP. Fascin, an actin bundling protein, has recently been shown to be a unique marker found in almost 100% of classical HL. METHODS: Because of the association of LyP with HL, fascin expression was analyzed by immunohistochemistry in LyP (n = 45), cutaneous CD30+ ALCL (n = 17) and pleomorphic T-cell lymphoma (n = 9) (PTL) and LyP associated with systemic lymphomas (7 HL, 2 ALCL, 1 MF), with the intent to determine if fascin expression can predict disease progression. RESULTS: Fascin was expressed by tumor cells in 11/45 (24%) cases of LyP, 11/17 (64%) cases of ALCL, 7/9 (77%) cases of PTL and 6/10 (60%) cases of LyP associated with systemic lymphomas. Fascin expression in LyP was significantly less frequent than in ALCL (p < 0.001) and also than in LyP associated with lymphomas (p < 0.05). There was no significant difference of fascin expression within the histological subtypes of LyP. We found no evidence of ALK expression nor of Epstein-Barr virus expression in any case either by in situ hybridization or immunohistochemistry in the LyP cases associated with HL. CONCLUSIONS: This is the first study to demonstrate that fascin is expressed in cutaneous CD30+ LPD and that it is a candidate marker of disease progression in LyP.

Biomarkers, Tumor↗

PubMed1994

Immunoregulatory abnormalities in patients with Epstein-Barr virus-associated B cell lymphoproliferative disorders.

EBVirus-associated B cell lymphoproliferative disorder (BLPD) is a recognized complication of primary immunodeficiency and organ as well as bone marrow transplantation. Although the nature of the immune defects that predispose to the development of BLPD are unknown, it is postulated that aberrant T cell responses are involved. It is our hypothesis that unbalanced lymphokine production is a major contributory factor to abnormal B cell growth in response to EBV, resulting in BLPD. Since IFN-alpha and IL-4 are important regulators of B cell proliferation and also regulate the synthesis of IgE, we determined serum levels of IFN-alpha, IL-4, and IgE in 8 patients with newly diagnosed BLPD. Comparison was made to healthy recipients of organ transplants on immunosuppressive therapy without BLPD, and normal EBV seropositive controls. Levels of serum IL-4 were significantly elevated in both patients with BLPD as well as in healthy immunosuppressed organ transplant recipients as compared with normal healthy individuals. Patients with BLPD exhibited a combination of significantly lower levels of serum IFN-alpha, and significantly higher levels of serum IgE than either healthy EBV seropositive individuals or healthy recipients of organ transplants on immunosuppressive therapy. These results suggest that imbalance in the proportions of circulating cytokines favoring B cell proliferation may be contributing to the development of EBV-associated BLPD. The potential significance of the finding of low IFN-alpha in patients who develop BLPD is exemplified by our recent success in the treatment of BLPD with IFN-alpha and intravenous IgG.

Adolescent↗

PubMed2001

Identification of prognostic factors in 61 patients with posttransplantation lymphoproliferative disorders.

PURPOSE: Prognostic studies of posttransplantation lymphoproliferative disorders (PTLDs) are hindered by the small number of cases at each transplant center. We analyzed prognostic factors and long-term outcome according to clinical manifestations, pathologic features, and treatment and investigated the prognostic value of the non-Hodgkin's lymphoma International Prognostic Index (IPI) in 61 patients with PTLD. PATIENTS AND METHODS: We studied 61 patients in two institutions who developed PTLD and analyzed factors influencing the complete remission and survival rates. RESULTS: In univariate analysis, factors predictive of failure to achieve complete remission were performance status (PS) > or = (P =.0001) and nondetection of Epstein-Barr virus (EBV) in the tumor (P =.01). Only a negative link with PS > or = 2 was observed in multivariate analysis. In univariate analysis, factors predictive of lower survival were PS > or = 2, the number of sites (one v > one), primary CNS localization, T-cell origin, monoclonality, nondetection of EBV, and treatment with chemotherapy. The IPI failed to identify a patient subgroup with better survival and was less predictive of the response rate than was a specific index using two risk factors (PS and number of involved sites), which defined three groups of patients: low-risk patients whose median survival time has not yet been reached, intermediate-risk patients with a median survival time of 34 months, and high-risk patients with a median survival time of 1 month. CONCLUSION: PS and the number of involved sites defined three risk groups in our population. The value of these prognostic factors needs to be confirmed in larger cohorts of patients treated in prospective multicenter studies.

Adolescent↗