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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.

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