Amyloid and Related Disorders: Surgical Pathology and by Maria M. Picken MD PhD FASN, Ahmet Dogan, Guillermo A.

By Maria M. Picken MD PhD FASN, Ahmet Dogan, Guillermo A. Herrera

The moment version of this article provides an summary of the latest advancements during this quarter together with scientific presentation, etiology, pathogenesis, and differential prognosis. the reason for varied treatments, together with transplantation, is mentioned and tissue prognosis (its pitfalls and methods for warding off them) and laboratory aid are integrated. The involvement of all significant organ platforms together with renal/genitourinary, cardiac, gastrointestinal, pulmonary, peripheral nerve/central apprehensive process, smooth tissue, dermis, lymph node/spleen and bone marrow pathology can be covered.

Amyloid and comparable issues, moment Edition may be worthy to really good and common pathologists in addition to cytopathologists. different doctors can also reap the benefits of this concise replace at the systemic amyloidoses.

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Additional info for Amyloid and Related Disorders: Surgical Pathology and Clinical Correlations

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Blood. 2006;108:2520–30. 2 Amyloid Diseases at the Molecular Level: General Overview and Focus on AL Amyloidosis 82. Gertz MA, Kyle RA, Greipp PR. The plasma cell labeling index: a valuable tool in primary systemic amyloidosis. Blood. 1989;74:1108–11. 83. Rajkumar SV, Gertz MA, Kyle RA. Primary systemic amyloidosis with delayed progression to multiple myeloma. Cancer. 1998;82:1501–5. 84. Gertz MA, Kyle RA, Noel P. Primary systemic amyloidosis: a rare complication of immunoglobulin M monoclonal gammopathies and Waldenstrom’s macroglobulinemia.

Similarly, the disease-associated Val30Met mutation of transthyretin shows significant differences in penetrance and clinical presentation among different ethnic groups and geographic areas [33]. Other factors, both environmental and genetic, are probably involved and understanding their roles will certainly improve our current knowledge of amyloidogenesis and, hopefully, pave the way to the discovery of novel therapeutic approaches. Common Constituents of Amyloid Deposits Amyloid deposits are classified and named based on the chemical nature of the most abundant fibril protein, according to internationally adopted nomenclature guidelines [34].

Specificity of abnormal assembly in immunoglobulin light chain deposition disease and amyloidosis. J Mol Biol. 1996;257:77–86. 132. Bellotti V, Merlini G. Toward understanding the molecular pathogenesis of monoclonal immunoglobulin light-chain deposition. Nephrol Dial Transplant. 1996;11:1708–11. 133. Raffen R, et al. Physicochemical consequences of amino acid variations that contribute to fibril formation by immunoglobulin light chains. Protein Sci. 1999;8:509–17. 134. Myatt EA, et al. Pathogenic potential of human monoclonal immunoglobulin light chains: relationship of in vitro aggregation to in vivo organ deposition.

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