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Blood, 15 March 2001, Vol. 97, No. 6, pp. 1885-1887

BRIEF REPORT

Acquired factor X deficiency in patients with amyloid light-chain amyloidosis: incidence, bleeding manifestations, and response to high-dose chemotherapy

Elie B. Choufani, Vaishali Sanchorawala, Timothy Ernst, Karen Quillen, Martha Skinner, Daniel G. Wright, and David C. Seldin

From the Section of Hematology and Oncology, Department of Medicine, Boston Medical Center; and the Amyloid Research and Treatment Program, Boston University School of Medicine, Boston, MA.

Acquired deficiency of factor X occurs in patients with systemic amyloid light-chain (AL) amyloidosis, presumably due to adsorption of factor X to amyloid fibrils. Of 368 consecutive patients with systemic AL amyloidosis evaluated at Boston Medical Center, 32 patients (8.7%) had factor X levels below 50% of normal. Eighteen of these patients (56%) had bleeding complications, which were more frequent and severe in the 12 patients below 25% of normal; 2 episodes were fatal. Ten factor X-deficient patients received high-dose melphalan chemotherapy followed by autologous stem cell transplantation. Of 7 patients alive 1 year after treatment, 4 had a complete hematologic response, and all 4 experienced improvement in their factor X levels. One of 2 additional patients with partial hematologic responses had improvement in factor X. Thus, aggressive treatment of the underlying plasma cell dyscrasia in AL amyloidosis can lead to the amelioration of amyloid-related factor X deficiency.

© 2001 by The American Society of Hematology.
 

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