Differential characteristics of Waldenström macroglobulinemia according to patterns of familial aggregation.
ABSTRACT Familial aggregation of Waldenström macroglobulinemia (WM) and related B-cell disorders (BCDs) suggests a role for genetic factors, but few data address environmental influences. We designed a questionnaire-based study to examine clinical and environmental factors in a cohort of WM families with various patterns of case aggregation. We analyzed data on 103 WM patients and 272 unaffected relatives from 35 multiple-case WM and 46 mixed WM/BCD kindred and 28 nonfamilial (sporadic) WM patients, using logistic regression models with generalized estimating equations to estimate odds ratios (ORs) and 95% confidence intervals (CIs) for association. In this study population, the WM disease process appeared similar among patients regardless of family history. Familial WM patients were more likely than unaffected relatives to report a history of autoimmune disease (OR, 2.27; 95% CI = 1.21-4.28) and infections (OR, 2.13; 95% CI = 1.25-3.64). Familial WM patients were also more likely to report exposure to farming (OR, 2.70; 95% CI = 1.34-5.42), pesticides (OR, 2.83; 95% CI = 1.56-5.11), wood dust (OR, 2.86; 95% CI = 1.54-5.33), and organic solvents (multiple-case WM OR, 4.21; 95% CI = 1.69-10.51) compared with unaffected family members. These data provide clues to both genetic and environmental factors that may influence development of WM. Well-designed case-control studies are needed to confirm these findings.
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ABSTRACT: Waldenström macroglobulinemia (WM) is a rare and currently incurable neoplasm of IgM-expressing B-lymphocytes that is characterized by the occurrence of a monoclonal IgM (mIgM) paraprotein in blood serum and the infiltration of the hematopoietic bone marrow with malignant lymphoplasmacytic cells. The symptoms of patients with WM can be attributed to the extent and tissue sites of tumor cell infiltration and the magnitude and immunological specificity of the paraprotein. WM presents fascinating clues on neoplastic B-cell development, including the recent discovery of a specific gain-of-function mutation in the MYD88 adapter protein. This not only provides an intriguing link to new findings that natural effector IgM(+)IgD(+) memory B-cells are dependent on MYD88 signaling, but also supports the hypothesis that WM derives from primitive, innate-like B-cells, such as marginal zone and B1 B-cells. Following a brief review of the clinical aspects and natural history of WM, this review discusses the thorny issue of WM's cell of origin in greater depth. Also included are emerging, genetically engineered mouse models of human WM that may enhance our understanding of the biologic and genetic underpinnings of the disease and facilitate the design and testing of new approaches to treat and prevent WM more effectively.ISRN hematology. 01/2013; 2013:815325.
Conference Paper: A novel theory of SAR image restoration and enhancement with ICA[Show abstract] [Hide abstract]
ABSTRACT: Active radar sensing is an important method of obtaining inventory information about remote and cloud-covered areas of the world. However, automatic interpretation of SAR images is often difficult due to speckle noise. Appearing as a random granular pattern, speckles seriously degrade the image quality and affect the task of human interpretation and scene analysis. For this kind of speckle removal problem, one of the difficulties is to overcome the tradeoff between noise reduction and preserving significant image details. In this paper, a novel theory of SAR image restoration and enhancement with independent component analysis (ICA) is proposed. We assume that the speckle noise in SAR images comes from a different signal source, which accompanies but is independent (their statistical characteristics are not same.) of the "true signal source" (image details). Thus the speckle removal problem can also he described as "signal source separation" problem. Then in order to enhance the "true signal source", we classify the basis images and span them into two different signal subspaces, namely "true signal subspace" and "speckle subspace". Finally we build different nonlinear estimators in each signal subspace to recover the original image. In our experiments, the SAR images consist of nine channels of images. We compare our method with two other well known speckle reduction approaches (Kuan filter and Lee filter). The results show that with our method the speckle noise is efficiently removed while at the same time important details (edges in particular) are retained without introducing artificial structures. We further calculate the ratio of standard deviation to mean (SD/Mean) for each image and use it as a criterion for image quality and find that the improvement with our method is more evident for images with ''high level speckle noise"Geoscience and Remote Sensing Symposium, 2004. IGARSS '04. Proceedings. 2004 IEEE International; 10/2004
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ABSTRACT: Waldenstrom macroglobulinemia is a distinct low-grade lymphoproliferative disease. There have been recent significant advances in understanding the underlying pathogenesis of this disease, including genetic and epigenetic regulators of tumor progression. Current studies have shown that the tumor microenvironment plays a critical role in cell proliferation, dissemination, and drug resistance. This review provides an update of the advances in the pathogenesis of factors both intrinsic (in the tumor clone) and extrinsic (in the bone marrow microenvironment) that regulate tumor progression in Waldenstrom macroglobulinemia. We next discuss novel agents that have been recently tested in clinical trials based on the advances observed in the pathogenesis of Waldenstrom macroglobulinemia.Current opinion in hematology 07/2011; 18(4):260-5. · 5.19 Impact Factor