Introduction Primary systemic light-chain (AL) amyloidosis is characterized by the extracellular deposition of amorphous amyloid fibrils derived from the N-terminal of the variable regions of monoclonal light chains that are produced by a plasma cell clone. 1 Amyloid deposi- tion in various tissues leads to organ dysfunction and systematic signs and symptoms; heart, kidneys, liver, and peripheral nervous system are most commonly affected. 2-4 Primary systemic light chain amyloidosis is an uncommon disease, and the diagnosis is often delayed or even missed because patients often present with nonspecific symptoms that might include fatigue, weakness, weight loss, dyspnea, postural hypotension, peripheral edema, paresthesias, and neuropathic pain, and only rarely with more specific signs such as tongue enlargement, periorbital purpura, and periarticular amyloid infiltration. 3 Survival of patients with AL amyloidosis is dependent on the pattern of organ and the number of affected organs; it is dominated by involvement of the heart, which is the major cause of death. 3-6 Treatment also depends on the pattern, number, and severity of organ involvement 6-9 and includes low-dose alkylators with ste- roids 10-12 or, for selected patients, high-dose melphalan (HDM) with autologous stem cell transplantation (ASCT). 13-19 Only Abstract Clinical Characteristics and Outcome of Primary Systemic Light-Chain Amyloidosis in Greece Michael Michael, Efstathios Kastritis, Sossana Delimpassi, Evridiki Michalis, Panagiotis Repoussis, Marie-Christine Kyrtsonis, Eirini Katodritou, Nicolaos Anagnostopoulos, Konstantinos Zervas, Meletios A. Dimopoulos, on behalf of the Greek Myeloma Study Group Background: Primary systemic light-chain (AL) amyloidosis is characterized by the deposition of immunoglobulin light chain–derived amyloid fibrils in various tissues leading to multiorgan dysfunction. Patients and Methods: In or- der to define characteristics, treatment, and outcome of Greek patients with AL amyloidosis, we analyzed 112 unse- lected patients with AL from several hospitals. Results: The heart was involved in 59% of patients and kidneys in 71%. Patients were treated with several different treatment regimens; high-dose dexamethasone-based regimens were used as primary treatment in 43% and melphalan-based regimens in 37%, while 12% received up-front bort- ezomib with dexamethasone. A hematologic response to first-line therapy was documented in 50% (complete re- sponse, 14.5%), and organ responses were observed in 25% of patients, the latter being strongly associated with achievement of hematologic response. Median overall survival was 34.2 months and was independently affected by heart involvement, creatinine, age, involvement of ≥ 2 organs, and bone marrow plasmacytosis > 30%. In patients with cardiac involvement, advanced age and extended bone marrow plasmacytosis were associated with an even worse outcome, while for patients without heart involvement, only bone marrow plasmacytosis was independently associated with survival. Hematologic response was associated with improved survival in patients with heart involve- ment but mostly in patients with less bone marrow infiltration. Conclusion: In this first series of patients from Greece with AL amyloidosis, disease features and outcome appeared similar to those reported from tertiary centers. Heart involvement and bone marrow plasma cell infiltration comprise adverse prognostic factors but also indicate the het- erogeneity of the disease and the need for individual treatment approaches. Clinical Lymphoma, Myeloma & Leukemia, Vol. 10, No. 1, 56-61, 2010; DOI: 10.3816/CLML.2010.n.006 Keywords: Autologous transplantation, Bortezomib, Melphalan, NT-proBNP, Thalidomide, Troponin Original Study Department of Clinical Therapeutics, University of Athens School of Medicine Greece Submitted: Jun 9, 2009; Revised: Jul 27, 2009; Accepted: Aug 12, 2009 Address for correspondence: Efstathios Kastritis, MD, Department of Clinical Therapeutics, University of Athens School of Medicine, 80 Vas Sofias Ave, 115 28, Athens, Greece Fax: 30-210-3381-511; e-mail: stathiskastritis@yahoo.com; ekastritis@gmail.com This summary may include the discussion of investigational and/or unlabeled uses of drugs and/or devices that may not be approved by the FDA. Electronic forwarding or copying is a violation of US and International Copyright Laws. Authorization to photocopy items for internal or personal use, or the internal or personal use of specific clients, is granted by CIG Media Group, LP, ISSN #2152-2650, provided the appropriate fee is paid directly to Copyright Clearance Center, 222 Rosewood Drive, Danvers, MA 01923 USA. www.copyright.com 978-750-8400. 56 | Clinical Lymphoma, Myeloma & Leukemia February 2010