Disease relevance of Lymphoproliferative Disorders

• It plays a critical role in immunity, as it is mutated in humans with X-linked lymphoproliferative syndrome (XLP), a fatal immunodeficiency characterized by an abnormal response to Epstein-Barr virus (EBV) infection [1].
• Castleman's disease is a lymphoproliferative disorder thought to be related to deregulated production of IL-6 [2].
• Here we show that injection of spleen cells from beta2-microglobulin-deficient (BALB/c x C57BL/6) F1 mice into BALB/c newborns with a disrupted beta2-microglobulin (beta2m) gene results in a lethal lymphoproliferative disorder associated with uncontrolled Th2 response, long-term persistence of donor B cells, and sustained blood eosinophilia [3].
• Angio-immunoblastic lymphadenopathy with dysproteinaemia (A.I.L.D.) is a lymphoproliferative disorder associated in one third of the patients with exposure to an allergen [4].
• A single recessive gene, lpr or gld, induces a lymphoproliferative disorder concomitant with autoimmune disease in mice [5].

Psychiatry related information on Lymphoproliferative Disorders

• Furthermore, the age-associated rise in IL-6 has been linked to lymphoproliferative disorders, multiple myeloma, osteoporosis, and Alzheimer's disease [6].

High impact information on Lymphoproliferative Disorders

• Our understanding of the X-linked lymphoproliferative syndrome (XLP) has advanced significantly in the last two years [7].
• Disruption of a new forkhead/winged-helix protein, scurfin, results in the fatal lymphoproliferative disorder of the scurfy mouse [8].
• Fas gene mutations in the Canale-Smith syndrome, an inherited lymphoproliferative disorder associated with autoimmunity [9].
• Lymphoproliferative disorders with early lethality in mice deficient in Ctla-4 [10].
• A similar human lymphoproliferative disorder also had an increase in c-myb expression [11].

Chemical compound and disease context of Lymphoproliferative Disorders

• Tumor necrosis factor sustains the generalized lymphoproliferative disorder (gld) phenotype [12].
• The incidence in children transplanted in the cyclosporine era is similar to that in adults, and the majority of tumors are lymphoproliferative disorders that often regress by reducing immunosuppression [13].
• Purine nucleoside analogs: emerging roles in indolent lymphoproliferative disorders [14].
• 2-Chlorodeoxyadenosine therapy in patients with T-cell lymphoproliferative disorders [15].
• Initial experience with AMSA as single agent treatment against malignant lymphoproliferative disorders [16].

Biological context of Lymphoproliferative Disorders

• GM-CSF and IL-2 induce specific cellular immunity and provide protection against Epstein-Barr virus lymphoproliferative disorder [17].
• To compare V kappa gene usage and the amount of somatic mutation in rearranged Ig genes from patients with lymphoproliferative disorders, we have polymerase chain reaction-amplified and sequenced a total of 26 V kappa genes from a total of 55 cases [18].
• Lack of the t(2;5) or other mutations resulting in expression of anaplastic lymphoma kinase catalytic domain in CD30+ primary cutaneous lymphoproliferative disorders and Hodgkin's disease [19].
• The importance of identifying BCR-coupled signaling networks and their cell cycle targets is underscored by the recognition that aberrant cell cycle control can lead to lymphoproliferative disorders or lymphoid malignancies [20].
• In this study of 198 DNA samples from 186 patients with a wide range of lymphoproliferative disorders (LPD), the methylation status of Myf-3 was assessed to evaluate its significance in the diagnosis of malignant LPD [21].

Anatomical context of Lymphoproliferative Disorders

• Unlike previous studies where constitutive expression of exogenous IL-6 genes resulted in lymphoproliferative disorders characterized by massive plasmacytosis, minimal plasma cell expansion occurred in the MSCV-IL-6 mice during the observation period [22].
• CTLA-4-deficient mice develop a fatal lymphoproliferative disorder, characterized by polyclonal expansion of peripheral lymphocytes [23].
• The possibility of exploiting an immunotherapeutic approach with IL-2/LAK cells in the management of chronic lymphoproliferative disorders does not gain support by these findings [24].
• Functional requirement for SAP in 2B4-mediated activation of human natural killer cells as revealed by the X-linked lymphoproliferative syndrome [25].
• Predictive factors for successful stem cell mobilization in patients with indolent lymphoproliferative disorders previously treated with fludarabine [26].

Gene context of Lymphoproliferative Disorders

• Constitutive activation of the Fas ligand gene in mouse lymphoproliferative disorders [27].
• Scurfy mice, which are deficient in a functional Foxp3, exhibit a severe lymphoproliferative disorder and display generalized over-production of cytokines [28].
• As in other lymphoproliferative disorders, the expression of CD38 or p53 or both was associated with a poorer survival probability [29].
• Despite Pten heterozygosity being associated with a lymphoproliferative disorder, we found no evidence of a hematopoietic defect in Pten(+/-) mice [30].
• In particular, CD40 was commonly observed on all B cells (both tumor and normal), whereas its ligand (CD40L), which is usually undetectable on resting normal B lymphocytes, was expressed in CLL and HCL but not in other chronic lymphoproliferative disorders [31].

Analytical, diagnostic and therapeutic context of Lymphoproliferative Disorders

• Administration of alemtuzumab, a humanized monoclonal antibody against CD52, to patients with lymphoproliferative disorders or as conditioning for hematopoietic stem cell transplantation resulted in a marked reduction in circulating lineage(-)HLA-DR(+) DCs (mean 31-fold reduction, P =.043) [32].
• Strong correlations between plasma IL-6 and plasma IL-10 with the HHV8 viral load suggest that both cytokines may be involved in the pathogenesis of this virus-associated lymphoproliferative disorder [33].
• These observations suggest that, in patients with RA who develop an EBV-associated lymphoproliferative disorder, a trial of discontinuation of immunosuppressive agents may be warranted before chemotherapy is considered [34].
• We used a denaturing gradient gel electrophoresis (DGGE) procedure with 40-nucleotide guanine- and cytosine-rich sequences in the polymerase chain reaction (PCR) and sequencing analysis to analyze the T cell antigen receptor (TCR)-Vgamma gene repertoire of infiltrating T lymphocytes in pulmonary lymphoproliferative disorders [35].
• Preventing acute rejection, Epstein-Barr virus infection, and posttransplant lymphoproliferative disorders after kidney transplantation: use of aciclovir and mycophenolate mofetil in a steroid-free immunosuppressive protocol [36].

References