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The Oncologist, Vol. 11, No. 7, 824-830, July 2006; doi:10.1634/theoncologist.11-7-824
© 2006 AlphaMed Press

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Lymphoma

Primary (AL) Amyloidosis in Plasma Cell Disorders

Antonia M. S. Müllera, Annette Geibelb, Hartmut P. H. Neumannc, Alexander Kühnemunda, Annette Schmitt-Gräffd, Joachim Böhmd, Monika Engelhardta

a Department of Hematology/Oncology, b Department of Cardiology/Angiology, c Department of Nephrology, and d Pathology Department, University Medical Center Freiburg, Freiburg, Germany

Key Words. AL amyloidosis • Proliferative plasma cell disorder • Light chain multiple myeloma

Correspondence: Monika Engelhardt, M.D., Department of Hematology/Oncology, University Medical Center Freiburg, Hugstetterstr. 55, D-79106 Freiburg, Germany. Telephone: 49-761-270-3401; Fax: 49-761-270-3318; e-mail: monika.engelhardt{at}uniklinik-freiburg.de

Primary (AL) amyloidosis is the most common form of systemic amyloidosis. The morbidity arises from extracellular deposition of immunoglobulin light chain (LC) fibrils in major organs, such as the kidneys, heart, and bowel. Organ dysfunction contributes to a high mortality and poor prognosis, with a median survival time of 1–2 years from diagnosis. Here, we present a 46-year-old man with an exceptional clinical course of an LC multiple myeloma with generalized amyloidosis, causing renal insufficiency, congestive heart failure, and complete intestinal necrosis. We have summarized recent knowledge on AL amyloidosis, its association with monoclonal gammopathies, clinical presentations, diagnostic tools, and treatment strategies. Our comprehensive overview of this rare and often fatal disease aims to increase the awareness of AL amyloidosis. This may facilitate earlier diagnosis, and thus allow initiation of prompt and specific therapies, which are indispensable in order to improve disease prognosis.






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