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Blood, 8 October 2009, Vol. 114, No. 15, pp. 3147-3157. Prepublished online as a Blood First Edition Paper on July 17, 2009; DOI 10.1182/blood-2009-04-202879. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2009-04-202879v1 114/15/3147    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via CrossRef Google Scholar Articles by Comenzo, R. L. PubMed PubMed Citation Articles by Comenzo, R. L. Related Collections How I Treat Free Research Articles Lymphoid Neoplasia Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  HOW I TREAT How I treat amyloidosis Raymond L. Comenzo1 1 Blood Bank, Stem Cell Processing Laboratory and Neely Cell Therapy Center, Tufts Medical Center, Boston, MA Amyloidosis is an uncommon disorder in which proteins change conformation, aggregate, and form fibrils that infiltrate tissues, leading to organ failure and death. The most frequent types are light-chain (AL) derived from monoclonal B-cell disorders producing amyloidogenic immunoglobulin light chains, and the hereditary and "senile systemic" (ATTR) variants from mutant and wild-type transthyretin (TTR). Diagnosis requires tissue biopsy. AL is more frequent and causes more organ disease than ATTR. Although both can cause cardiomyopathy and heart failure, AL progresses more quickly, so survival depends on timely diagnosis. Typing is usually based on clinical and laboratory findings with monoclonal gammopathy evaluation and, if indicated, TTR gene testing. Direct tissue typing is required when one patient has 2 potential amyloid-forming proteins. In coming years, widespread use of definitive proteomics will improve typing. New therapies are in testing for ATTR, whereas those for AL have followed multiple myeloma, leading to improved survival. Challenges of diagnosing and caring for patients with amyloidosis include determination of type, counseling, and delivery of prompt therapy often while managing multisystem disease. Recent advances grew from clinical research and advocacy in many countries, and global husbandry of such efforts will reap future benefits for families and patients with amyloidosis. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

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