Table 4.

Main studies investigating coagulation abnormalities in patients with AL‐amyloidosis since 2000

Reference	Study design	Number of patients/sex ratio (male/female)	Median age (years)	Coagulation abnormalities a and relationships with clinical features
Mumford AD et al., 2000 41	Retrospective single‐centre cohort study	337/0.54	61.2	‐TT prolongation (32% of patients) associated with hepatic amyloid deposits (P < 10−4), 24‐h proteinuria (P < 10−3), and hypoalbuminaemia (P < 10−5) ‐PT prolongation (24% of patients) associated with abnormal bleeding (P 0.0012) ‐aPTT prolongation (14% of patients) ‐FX:C deficiency (<70 IU/dL): 22/154 (14%), of whom 7 (5%) < 20 IU/dL ‐FX:Ag/FX:C 2.5 in patients with FX deficiency vs FX:Ag/FX:C 0.96 in patients without (P < 10−4) ‐Mild FVII:C deficiency in 2 patients (44 and 23 IU/dL) ‐Absence of FX inhibitor
Gamba G et al., 2000 42	Prospective	36/2.0	NA	‐TT prolongation (85% of patients) ‐PT prolongation (22% of patients) ‐aPTT prolongation (65% of patients) ‐ FX:C deficiency (< 65 IU/dL)(27% of patients)
Choufani EB et al., 2001 43	Prospective clinical trial	368/1.5 b	58.0 b	‐FX:C deficiency (< 50 IU/dL): 32/368 (8.7% a ) of whom 12 < 25 IU/dL (9 with bleeding complications) ‐Frequency and severity worse in the patients with the lowest levels of FX
Patel G et al., 2019 44	Retrospective single‐centre cohort study	104/0.54	63.4	‐FX:C deficiency (<50 IU/dL): 10/104 (9.6% a ) of whom 2 < 25 IU/dL

^a Patients receiving vitamin K antagonist were excluded.

^b Sex ratio and median age of patients with FX deficiency; TT: thrombin time.

aPTT: activated partial prothrombin time; Ag, antigen; F, factor; FX:C, FX clotting activity; NA, non‐available; PT, prothrombin time.