TY - JOUR
T1 - Systemic AL amyloidosis with acquired factor X deficiency
T2 - A study of perioperative bleeding risk and treatment outcomes in 60 patients
AU - Thompson, Carrie A.
AU - Kyle, Robert
AU - Gertz, Morie
AU - Heit, John
AU - Pruthi, Rajiv
AU - Pardanani, Animesh
PY - 2010/3
Y1 - 2010/3
N2 - Systemic light-chain (AL) amyloidosis may be associated with acquired factor X (FX) deficiency and optimal management of this coagulopathy is unknown. We reviewed our experience with 60 patients with isolated FX deficiency (≤50%) due to AL amyloidosis that underwent an invasive procedure between 1975 and 2007. They were classified as having severe (<10%; n = 6), moderate (10-25%; n = 15), or mild (26-50%; n = 39) FX deficiency. The patients underwent a total of 112 procedures, 19 (17%) of which were managed with periprocedural treatment with one or more hemostatic agents. There were complications in 14 (13%) procedures (bleeding = 12, thrombosis = 1, death = 1). Baseline FX level was not predictive of bleeding risk; the only association with postintervention bleeding was central venous catheter placement. However, bleeding complications were relatively infrequent, particularly in patients with mild or moderate FX deficiency undergoing nonvascular procedures. Activated recombinant factor VII might be considered in patients undergoing major surgical procedures, but further experience is needed. Optimal management of AL patients with FX deficiency undergoing invasive procedures remains to be determined.
AB - Systemic light-chain (AL) amyloidosis may be associated with acquired factor X (FX) deficiency and optimal management of this coagulopathy is unknown. We reviewed our experience with 60 patients with isolated FX deficiency (≤50%) due to AL amyloidosis that underwent an invasive procedure between 1975 and 2007. They were classified as having severe (<10%; n = 6), moderate (10-25%; n = 15), or mild (26-50%; n = 39) FX deficiency. The patients underwent a total of 112 procedures, 19 (17%) of which were managed with periprocedural treatment with one or more hemostatic agents. There were complications in 14 (13%) procedures (bleeding = 12, thrombosis = 1, death = 1). Baseline FX level was not predictive of bleeding risk; the only association with postintervention bleeding was central venous catheter placement. However, bleeding complications were relatively infrequent, particularly in patients with mild or moderate FX deficiency undergoing nonvascular procedures. Activated recombinant factor VII might be considered in patients undergoing major surgical procedures, but further experience is needed. Optimal management of AL patients with FX deficiency undergoing invasive procedures remains to be determined.
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U2 - 10.1002/ajh.21603
DO - 10.1002/ajh.21603
M3 - Article
C2 - 20052750
AN - SCOPUS:77249130849
SN - 0361-8609
VL - 85
SP - 171
EP - 173
JO - American journal of hematology
JF - American journal of hematology
IS - 3
ER -