By Per Westermark M.D., Ph.D. (auth.), Maria M. Picken MD, PhD, FASN, Ahmet Dogan, M.D., Ph.D., Guillermo A. Herrera, M.D. (eds.)
Abnormal proteins are identified to be linked to a number of pathologies. so much particularly, those contain amyloidoses, monoclonal protein deposits linked to plasma mobilephone dyscrasia/multiple myeloma, cryoglobulins and diverse comparable geared up and non-organized deposits.
Amyloid and similar Disorders provides an summary of the main
recent advancements during this sector together with medical presentation, etiology, pathogenesis, and differential analysis. the reason for numerous remedies, together with transplantation, is mentioned and tissue prognosis (its pitfalls and methods for heading off them) and laboratory aid are incorporated. The involvement of all significant organ structures together with renal/genitourinary, cardiac, gastrointestinal, pulmonary, peripheral nerve/central apprehensive process, gentle tissue and bone marrow pathology is roofed. This process offers a unifying idea of those pathologic approaches, that have systemic involvement, and that have, hitherto, now not been universally liked. expertise of those illnesses between a much broader viewers of pathologists may well elevate the speed in their prognosis in addition to that of previous prognosis. This quantity may be worthwhile to really good and normal pathologists in addition to cytopathologists; different doctors can also take advantage of this concise replace at the systemic amyloidoses.
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Additional info for Amyloid and Related Disorders: Surgical Pathology and Clinical Correlations
Blood. 2003;101:827–30. 94. Dispenzieri A, Lacy MQ, Katzmann JA, et al. Absolute values of immunoglobulin free light chains are prognostic in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation. Blood. 2006;107:3378–83. 95. Wechalekar A et al. A new staging system for AL amyloidosis incorporating serum free light chains, cardiac troponin-T and NT-proBNP. Blood 2009;114:abstr. 2796. 96. Kumar S et al. A novel prognostic staging system for light chain amyloidosis (AL) incorporating markers of plasma cell burden and organ involvement.
Utility of Doppler myocardial imaging, cardiac biomarkers, and clonal immunoglobulin genes to assess left ventricular performance and stratify risk following peripheral blood stem cell transplantation in patients with systemic light chain amyloidosis (Al). J Am Soc Echocardiogr. 2011;24:444–54. M. Nuvolone et al. 113. Perfetti V, Ubbiali P, Vignarelli MC, et al. Evidence that amyloidogenic light chains undergo antigendriven selection. Blood. 1998;91:2948–54. 114. Abraham RS, Geyer SM, Ramirez-Alvarado M, et al.
3 AA Amyloidosis Amanda K. Ombrello and Ivona Aksentijevich Keywords AA amyloidosis • Serum amyloid A • Rheumatic disease • Autoinflammatory disease • IL-1-mediated disease • Cryopyrin-associated periodic fever syndromes • Familial Mediterranean fever • Tumor necrosis factor receptor-associated periodic syndrome • Familial cold autoinflammatory syndrome • Muckle–Wells syndrome • Neonatal onset multisystem inflammatory disease • Hyper IgD syndrome • Inflammatory bowel disease • Biologic medications AA amyloidosis, also known as reactive amyloidosis, is a form of amyloidosis that develops in patients with chronic inflammatory states.
Amyloid and Related Disorders: Surgical Pathology and Clinical Correlations by Per Westermark M.D., Ph.D. (auth.), Maria M. Picken MD, PhD, FASN, Ahmet Dogan, M.D., Ph.D., Guillermo A. Herrera, M.D. (eds.)