TY - JOUR
T1 - Resolution of Factor X Deficiency in Primary Amyloidosis Following Splenectomy
AU - Rosenstein, Elliot D.
AU - Itzkowitz, Steven H.
AU - Penziner, Alan S.
AU - Cohen, Jonathan I.
AU - Mornaghi, Ricardo A.
PY - 1983/3
Y1 - 1983/3
N2 - A 57-year-old man with primary amyloidosis was initially seen with hematuria, cutaneous bleeding, and hepatosplenomegaly. Factor X was determined to be 10% to 16% of normal plasma values. Administration of vitamin K-dependent factor concentrate transiently improved in vitro clotting tests but did not alter the clinical course. Following a splenectomy, bleeding ceased and factor X levels returned to normal, remaining so despite discontinuation of factor concentrate infusion. Amyloid fibrils extracted from the patient's spleen were determined to be derived from λV1 light chains. The importance of splenectomy as an effective therapeutic modality is discussed.
AB - A 57-year-old man with primary amyloidosis was initially seen with hematuria, cutaneous bleeding, and hepatosplenomegaly. Factor X was determined to be 10% to 16% of normal plasma values. Administration of vitamin K-dependent factor concentrate transiently improved in vitro clotting tests but did not alter the clinical course. Following a splenectomy, bleeding ceased and factor X levels returned to normal, remaining so despite discontinuation of factor concentrate infusion. Amyloid fibrils extracted from the patient's spleen were determined to be derived from λV1 light chains. The importance of splenectomy as an effective therapeutic modality is discussed.
UR - http://www.scopus.com/inward/record.url?scp=84948721086&partnerID=8YFLogxK
U2 - 10.1001/archinte.1983.00350030211041
DO - 10.1001/archinte.1983.00350030211041
M3 - Article
C2 - 6830397
AN - SCOPUS:84948721086
SN - 0003-9926
VL - 143
SP - 597
EP - 599
JO - Archives of Internal Medicine
JF - Archives of Internal Medicine
IS - 3
ER -